Document:

EX-10.2

 Exhibit 10.2 

[*] = Certain confidential information contained in this document, marked by brackets, is omitted because it is both (i) not material and
(ii) would be competitively harmful if publicly disclosed. 
 UNIVERSITY OF MIAMI 

EXCLUSIVE LICENSE AGREEMENT 
 This
Exclusive License Agreement (this “Agreement”) is entered into and made effective as of the last dated signature below (the “Effective Date”) between University of Miami, a Florida not-for-profit corporation, having business offices at 1951 NW 7th Avenue, (C234), Miami, Florida 33136 (“UNIVERSITY”), on behalf of
itself, Emory and MGH (as defined below), and Orphazyme ApS, CVR no. DK 3226 6355, a for-profit corporation organized under the laws of Denmark, having business offices at Ole Maaloes Vej 3, DK-2200 Copenhagen N, Denmark (“LICENSEE”). For purposes of this Agreement, each of UNIVERSITY and LICENSEE may be individually referred to as a “Party” and collectively referred to
as the “Parties.” 
 BACKGROUND 

Certain Technology and Know-How (as defined below) was developed during research conducted by investigators at the
UNIVERSITY, Emory University (“Emory”), and The General Hospital Corporation, d/b/a Massachusetts General Hospital (“MGH”), collectively the “Institutions”. Dr. Michael Benatar currently at
UNIVERSITY and formerly at Emory, in collaboration with Drs. Merit Cudknowicz, Nazem Atassi, and David Schoenfeld at MGH have developed certain Technology and Know-How described in Appendix A. Each of the
investigators have assigned the Technology and Know-How licensed hereunder to their respective Institution in accordance with their Institution’s Intellectual Property policy, including all right and
title to certain inventions as described in the investigators’ invention disclosure documents attached as Appendix A. 
 The Technology and Know-How is jointly owned by the Institutions. Pursuant to a certain Three-Way Inter-Institutional Agreement (the “Three-Way
Inter-Institutional Agreement”) effective on March 5, 2019 and executed between the Institutions, Emory and MGH have granted the UNIVERSITY the exclusive right to negotiate and administer an exclusive license to each of Emory’s
and MGH’s rights in the Technology and Know-How being licensed to LICENSOR hereto. 
 Each of the Institutions
want to have the Technology and Know-How perfected and marketed as soon as possible so that resulting products may be available for public use and benefit. LICENSEE wants to acquire an exclusive license to the
Technology and Know-How for the purposes of exploiting Licensed Products and Licensed Processes and practicing the invention(s) disclosed, in the Territory and in the Field of Use as set forth and defined
below. 
 NOW, THEREFORE, in consideration of the premises and mutual covenants herein contained, and for other good and valuable consideration, the receipt
and sufficiency of which are hereby acknowledged, the Parties hereto agree as follows: 
 AGREEMENT 

 

	 	1.	 DEFINITIONS 

  

	 	1.1	 “Affiliate” shall mean any corporation or other business entity controlled by, controlling or
under common control with UNIVERSITY or LICENSEE. For this purpose, “control” shall mean direct or indirect beneficial ownership of at least a fifty percent (50%) of the equity interests of, or at least a fifty percent (50%) interest in
the income of such corporation or other business entity, or any business entity that is more than fifty percent (50%) owned by a business entity that owns more than fifty percent (50%) of LICENSEE, or such other relationship as in fact, constitutes
actual control. 

  
 1 

	 	1.2	 “Amyotrophic Lateral Sclerosis” shall mean amyotrophic lateral sclerosis (ALS), as broadly
defined, to include patients with mixed upper motor neuron (UMN) and lower motor neuron (LMN) pathology (classic ALS), primarily LMN disease (LMN-predominant ALS and progressive muscular atrophy) and primarily
UMN disease (UMN-predominant ALS and primary lateral sclerosis). 

  

	 	1.3	 “Arimoclomol” shall mean Arimoclomol or any derivative of Arimoclomol exhibiting the same
biological activity and for which the data generated under the Study is used to support an application to the FDA or similar regulatory authority in order to gain Regulatory Approval. 

 

	 	1.4	 “Control” shall mean, with respect to any intellectual property right, the possession of the
right (whether by ownership, license or otherwise (other than pursuant to a license granted under this Agreement)), to assign, or grant a license, sublicense or other right to or under, such intellectual property right as provided for herein without
violating the terms of any agreement or other arrangement with any third party. 

  

	 	1.5	 “Excluded Entity” shall mean any corporation, business entity or person (i) associated
with the development or commercialization of alcohol, tobacco products or private prisons, military armaments and pornography or (ii) on any list of prohibited individuals or entities enacted under United States economic sanctions and
anti-boycott laws. 

  

	 	1.6	 “Field of Use” shall mean any and all uses or applications of Institutions’ intellectual
property titled “Arimoclomol for SOD1 ALS” with University’s reference number identified as UMIP- 138, Emory’s reference number identified as 08029 and MGH’s reference number identified as 25521.

  

	 	1.7	 “Indication” means any human disease, condition or syndrome, or sign or symptom of, or
associated with, a human disease, condition or syndrome in a particular target patient population. 

  

	 	1.8	 “Inventors” shall mean Dr. Michael Benatar during the term of his employment at
UNIVERSITY and Emory, in addition to Drs. Merit Cudknowicz and Nazem Atassi during the term of their respective employments at MGH. 

  

	 	1.9	 “Know-How” shall mean all (a) information,
techniques, technology, practices, trade secrets, inventions (whether patentable or not), methods, knowledge, skill, experience, data, results (including pharmacological, toxicological and clinical test data and results, chemical structures,
sequences, processes, formulae, techniques, research data, reports, standard operating procedures and batch records), analytical and quality control data, analytical methods (including applicable reference standards), full batch documentation,
packaging records, release, stability, storage and shelf-life data, and manufacturing process information, results or descriptions, software and algorithms of each of the Institutions; and (b) tangible manifestations thereof. As used in this
Agreement, “clinical test data” shall include all information related to clinical or non-clinical testing, including patient report forms, investigators’ reports, biostatistical,
pharmaco-economic and other related analyses, regulatory filings and communications, de-identified of any individually identifiable health information, including any data that contains one or more of the
identifiers listed in §164.514(b)(2) and in compliance with any federal and state privacy laws, including the Health Insurance Portability and Accountability Act of 1996, as amended (“HIPAA”). The Parties understand and agree
that this Agreement will not include any patient data for commercial use which has not been de-identified as contemplated above. 

  
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[*] = Certain confidential information contained in this document, marked by brackets, is omitted because it is both (i) not material and (ii) would be
competitively harmful if publicly disclosed. 

	 	1.10	 “Licensed Product” shall mean any pharmaceutical product containing Arimoclomol, which is
made, developed, used or sold by LICENSEE or an Affiliate or Sublicensee of LICENSEE for which the applicable Indication is intended to receive Regulatory Approval with a product label specifying the treatment of patients with Amyotrophic Lateral
Sclerosis (the “Approved ALS Indication”); and is intended to be the subject of a Regulatory Approval filing for the Approved ALS Indication which includes the clinical test data and results from the Study. 

 

	 	1.11	 “Licensed Process” shall mean, solely for Arimoclomol, any process or part thereof made,
developed, used or sold by LICENSEE or an Affiliate or Sublicensee of LICENSEE which incorporates or uses the Technology. 

  

	 	1.12	 “Net Sales” shall be calculated as set forth in this section, and shall mean gross amounts
invoiced by LICENSEE and/or its Affiliates and/or Sublicensees on commercial sales of Licensed Products or use of Licensed Processes after Regulatory Approval, if applicable, thereof to third parties (excluding Sublicensees and Affiliates), less
deductions for the following, determined in accordance with generally accepted accounting principles, consistently and strictly applied: 

  

	 	a.	 sales and excise taxes (including annual fees due under Section 9008 of the United States Patient
Protection and Affordable Care Act of 2010 (Pub. L. No. 111-48) and other comparable Laws as and to the extent applicable), value added taxes, and duties which fall due and are paid by the purchaser as a
direct consequence of such sales and any other governmental charges imposed upon the importation, use or sale of Licensed Products or Licensed Processes, but only to the extent that such taxes and duties are actually included and itemized in the
gross sales amounts invoiced to and specifically paid by the purchaser over and above the price of the Licensed Products or Licensed Processes; 

  

	 	b.	 trade, quantity and cash discounts actually allowed and taken; 

 

	 	c.	 allowances or credits to customers on account of shelf adjustments, failure to supply, rejection, withdrawal,
recall or return of Licensed Products or on account of retroactive price reductions affecting Licensed Products, to the extent that such allowances or credits are actually allowed and taken; 

 

	 	d.	 any charges for freight, postage, shipping or transportation or for shipping insurance; 

 

	 	e.	 rebates and charge backs specifically related to Licensed Products or Licensed Processes on an actual credited
or paid basis, including those granted to government agencies and group purchasing and managed care organizations and entities (such rebates and charge backs to be accrued as an estimate in the month in which the related Licensed Products or
Licensed Processes are sold by using generally accepted accounting principles) to the extent that such rebates and charge backs are actually allowed and taken; 

 

	 	f.	 sales contract administrative fees, fees paid to distributors, wholesaler fees or service charges and other
payments to customers in connection with the sale of Licensed Products or Licensed Processes for any non-separable services, to the extent actually allowed and taken; and 

If a Licensed Product is a Combination Product (as defined below), the Net Sales for such Combination Product in each country or jurisdiction
shall be calculated as follows: 
 (i) If LICENSEE or any of its Affiliates or Sublicensees separately sells in such country or other
jurisdiction, (A) a product containing as its sole active ingredient Arimoclomol contained in such Combination Product (the “Mono Product”) and (B) products containing as their sole active ingredients the other active
ingredients in such Combination Product, the Net Sales attributable to such Combination Product shall be calculated by multiplying actual Net Sales of such Combination Product by the fraction A/(A+B) where: “A” is LICENSEE’s (or its
Affiliate’s or Sublicensee’s, as applicable) average Net Sales price 

  
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[*] = Certain confidential information contained in this document, marked by brackets, is omitted because it is both (i) not material and (ii) would be
competitively harmful if publicly disclosed. 

 
during the period to which the Net Sales calculation applies for the Mono Product in such country or other jurisdiction and “B” is LICENSEE’s (or its Affiliate’s or
Sublicensee’s, as applicable) average net sales price (determined in the same manner as “Net Sales”) during the period to which the Net Sales calculation applies in such country or other jurisdiction, for products that contain
as their sole active ingredients the other active ingredients in such Combination Product. 
 (ii) If LICENSEE or any of its Affiliates or
Sublicensees separately sells in such country or other jurisdiction the Mono Product but does not separately sell in such country or other jurisdiction products containing as their sole active ingredients the other active ingredients in such
Combination Product, the Net Sales attributable to such Combination Product shall be calculated by multiplying the Net Sales of such Combination Product by the fraction A/C where: “A” is LICENSEE’s (or its Affiliate’s or
Sublicensee’s, as applicable) average Net Sales price during the period to which the Net Sales calculation applies for the Mono Product in such country or other jurisdiction, and “C” is LICENSEE’s (or its Affiliate’s or
Sublicensee’s, as applicable) average Net Sales price in such country or other jurisdiction during the period to which the Net Sales calculation applies for such Combination Product. 

(iii) If LICENSEE and its Affiliates and Sublicensees do not separately sell in such country or other jurisdiction the Mono Product but do
separately sell products containing as their sole active ingredients the other active ingredients contained in such Combination Product, the Net Sales attributable to such Combination Product shall be calculated by multiplying the Net Sales of such
Combination Product by the fraction (D-E)/D where: “D” is the average Net Sales price during the period to which the Net Sales calculation applies for such Combination Product in such country or
other jurisdiction and “E” is the average net sales price (determined in the same manner as “Net Sales”) during the period to which the Net Sales calculation applies for products that contain as their sole active
ingredients the other active ingredients in such Combination Product. 
 (iv) If LICENSEE and its Affiliates and Sublicensees do not
separately sell in such country or other jurisdiction both the Mono Product and the other active ingredient or ingredients in such Combination Product, the Net Sales attributable to such Combination Product shall be determined by the Parties in good
faith based on the relative fair market value of such Mono Product and such other active ingredient or ingredients. If the Parties cannot agree on such relative value, the Dispute shall be resolved pursuant to Section 14.4. Net Sales of a
Licensed Product shall only include sales of the Licensed Product containing Arimoclomol, and not any other compound (other than a compound that is sold as a fixed-dosed combination or co-packed with
Arimoclomol) or therapy that may be used in combination with such Licensed Product. 
 As used in this definition of “Net
Sales,” “Combination Product” means any Licensed Product sold together (whether or not as part of a kit) with any other active pharmaceutical ingredient or medical device or component thereof. 

 

	 	1.13	 “Regulatory Approval” means the approval, license or authorization of the U.S. Food and Drug
Administration (“FDA”) or similar regulatory body in another country or region necessary for the marketing and sale of a product for a particular Indication in the United States, the European Union, Japan, Brazil, Australia, New
Zealand, China, Mexico, Korea, Canada, the United Kingdom, and India. 

  

	 	1.14	 “Study” shall mean the Arimoclomol clinical trial (ClinicalTrials.gov Identifier: NCT00706147;
entitled: Phase II/III Randomized, Placebo-controlled Trial of Arimoclomol in SOD1 Positive Familial Amyotrophic Lateral Sclerosis), which includes the study referred to in the definition of “Licensed Technology” in the Three-Way Inter-Institutional Agreement. 

  
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[*] = Certain confidential information contained in this document, marked by brackets, is omitted because it is both (i) not material and (ii) would be
competitively harmful if publicly disclosed. 

	 	1.15	 “Sublicensee” shall mean any third party to whom LICENSEE has granted a license to make, have
made, use and/or sell Licensed Product or Licensed Process under the Technology (a “Sublicense”). 

  

	 	1.16	 “Technology” shall mean all proprietary information known by the Inventors on or before the
Effective Date, reasonably necessary to practice or use the Know-How relating to the Study, including but not limited to, information, data, techniques, biological materials, methods, protocols, and the like,
to the extent they exist or have been developed on or before the Effective Date. 

  

	 	1.17	 “Territory” shall mean the world. 

 

	 	2.	 GRANT 

  

	 	2.1	 LICENSE GRANTS. 

  

	 	a.	 The UNIVERSITY hereby grants to LICENSEE and LICENSEE hereby accepts an exclusive license in the Territory for
the Field of Use, under the Technology and Know-How to research, develop, make, have made, use, sell and import the Licensed Products and/or Licensed Processes. 

 

	 	b.	 Subject to Section 2.2 and 2.3, the UNIVERSITY hereby grants to LICENSEE an exclusive, worldwide license
in the Field of Use to the Technology to research, develop, make, have made, use, sell and import Licensed Products and/or Licensed Processes. 

  

	 	2.2	 RETAINED RIGHTS. Each of the Institutions reserve and retain the right to use and/or license all Know-how, Technology or Study data (“Intellectual Property” ) for the purposes of research and understanding the natural history of SOD1+ ALS and/or for Institutions’ own internal educational
and non-commercial research purposes. Additionally, Institutions reserve and retain a non-exclusive right to license any Intellectual Property relating to the placebo
data to any third parties for commercial or non-commercial purposes; it being understood and agreed that UNIVERSITY, by and on behalf of the Institutions, shall notify LICENSEE within [*] calendar days of the
identity of any licensee (and sublicensee thereof) of such placebo data. Institutions will be free to publish any results of such assessment or research after providing LICENSEE with a [*] day period in which to review each publication to identify
any disclosure of LICENSEE’s proprietary information. Upon LICENSEE’s reasonable request, Institutions will delete references to LICENSEE’s proprietary information in any paper or presentation. Notwithstanding anything contrary
herein, ALS Association (one of the sponsors of the Study) has a non-exclusive, royalty free, worldwide license to practice all Technology including access to and the right to use biological materials, with
the right to grant sublicenses to others, solely for non-commercial research and development purposes. 

  

	 	2.3	 GOVERNMENT RESERVATION. Rights under this Agreement are subject to rights required to be granted to the federal
government of the United States of America (the “Government”) pursuant to Title 35 U.S.C. Section 200-212, including a nonexclusive, nontransferable, irrevocable, paid-up license to practice or have practiced for or on behalf of the United States the subject inventions throughout the world. 

 

	 	2.4	 SUBLICENSING. 

  

	 	a.	 Subject to the terms and conditions of this Section 2.4 and otherwise as set forth in this Agreement,
LICENSEE may grant Sublicenses (through multiple tiers) to third parties from time to time; provided, that, solely for any Sublicense to an Excluded Entity, LICENSEE has requested and obtained the prior written approval of UNIVERSITY. Each
Sublicensee shall agree in writing with LICENSEE to accept the conditions and restrictions 

  
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[*] = Certain confidential information contained in this document, marked by brackets, is omitted because it is both (i) not material and (ii) would be
competitively harmful if publicly disclosed. 

	 	
agreed to by LICENSEE in this Agreement. Sublicenses with any exclusivity must include diligence requirements commensurate with the diligence requirements under this Agreement. Any agreement
granting a Sublicense shall state that the Sublicense is subject to the termination of this Agreement. LICENSEE shall have the same responsibility for the activities of any Sublicensee as if the activities were directly those of LICENSEE.

  

	 	b.	 For clarity, royalties on Net Sales negotiated and agreed upon under this Agreement are
“pass-through” by nature. An earned royalty is calculated as a percentage of Net Sales of Licensed Products and/or Licensed Processes of LICENSEE or Sublicensee(s). 

 

	 	c.	 LICENSEE shall provide UNIVERSITY with a copy of each Sublicense agreement and any other agreement which
transfers intellectual property rights granted hereunder to a third party (other than routine research and development agreements, such as agreements with contract research organizations), within [*] days following the execution of the Sublicense
agreement. 

  

	 	d.	 Notwithstanding the Sublicensee’s payment obligation to LICENSEE, LICENSEE shall be directly responsible
for all royalties and payments due pursuant to Section 3. 

  

	 	3.	 ROYALTIES AND OTHER CONSIDERATION 

 

	 	3.1	 CONSIDERATION. In consideration of the licenses herein granted, LICENSEE shall pay fees and royalties to
UNIVERSITY as follows: 

  

	 	a.	 License issue fee of seventy-five thousand U.S. dollars (U.S. $75,000) is due to UNIVERSITY within [*] days of
the Effective Date of this Agreement. 

  

	 	b.	 On and after such time as the Licensed Product has received Regulatory Approval for the Approved ALS
Indication, running royalty on licensed Technology is calculated as a percentage equal to three-quarters of one percent (0.75%) of the annual Net Sales of the Licensed Product(s) or Licensed Process(es) which use the Technology.

  

	 	c.	 By the first (1st) day of each anniversary specified below
of the Effective Date and until the fourteenth (14th) anniversary of the Effective Date or the earlier termination of this Agreement, LICENSEE agrees to pay UNIVERSITY an annual fee of: 

 

	 	(i)	 [*] on the [*] anniversary; 

 

	 	(ii)	 [*] on the [*] anniversary; 

 

	 	(iii)	 [*] on the [*] anniversary; 

 

	 	(iv)	 [*] on the [*] through [*] anniversaries; and 

 

	 	(v)	 [*] on the [*] anniversary and every anniversary thereafter until the fourteenth (14th) anniversary of
the Effective Date or the earlier termination of this Agreement. 

 Each such annual fee is fully creditable towards any
other consideration, including royalty and milestone payments that are, as set forth herein, due to the UNIVERSITY by LICENSEE during any calendar in which such annual fee is paid. 

 

	 	d.	 Royalties are payable on a Licensed
Product-by-Licensed Product or Licensed Process-by-Licensed Process and country-by-country basis beginning on the date of first commercial sale of such Licensed Product or Licensed Process in such country and ending on the termination of the Term.

  
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[*] = Certain confidential information contained in this document, marked by brackets, is omitted because it is both (i) not material and (ii) would be
competitively harmful if publicly disclosed. 

	 	3.2	 CURRENCY. All payments hereunder shall be made in U.S. dollars. For converting any amounts made in a currency
other than U.S. dollars, the Parties will use the conversion rate customarily used by the LICENSEE or Sublicensee at such time for preparation of its relevant financial statements. 

 

	 	3.3	 PAYMENT TERMS. All payments due hereunder are payable by check or wire transfer to the address listed in
Section 13 or using the wiring instructions provided by UNIVERSITY, as applicable, and shall be deemed received when the complete payment is credited to UNIVERSITY’s bank account. All payments shall be made to the UNIVERSITY, which shall
be responsible for payments to Emory and MGH in accordance with the Three-Way Inter-Institutional Agreement. Until all funds are received by UNIVERSITY, the payment by LICENSEE is not considered to be
complete. No transfer, exchange, collection or other charges, including any wire transfer fees, shall be deducted from such payments. 

  

	 	3.4	 WITHHOLDING TAXES. Each Party shall be entitled to deduct and withhold from any amounts payable under this
Agreement such taxes as are required to be deducted or withheld therefrom under any provision of applicable Law. The Party that is required to make such withholding (the “Paying Party”) will: (i) deduct those taxes from such
payment, (ii) timely remit the taxes to the proper taxing authority, and (iii) send evidence of the obligation together with proof of tax payment to the recipient Party (the “Payee Party”) on a timely basis following that
tax payment; provided, however, that before making any such deduction or withholding, the Paying Party shall give the Payee Party notice of the intention to make such deduction or withholding (and such notice, which shall set forth in reasonable
detail the authority, basis and method of calculation for the proposed deduction or withholding, shall be given at least a reasonable period of time before such deduction or withholding is required, in order for such Payee Party to obtain reduction
of or relief from such deduction or withholding). Each Party agrees to cooperate with the other Parties in claiming refunds or exemptions from, or reductions in, such deductions or withholdings under any applicable Law or treaty to ensure that any
amounts required to be withheld pursuant to this Section 3.4 are reduced to the fullest extent permitted by applicable Law. In addition, the Parties shall cooperate to minimize indirect taxes (such as value added tax, sales tax, consumption tax
and other similar taxes) in connection with this Agreement, as applicable. 

  

	 	3.5	 TAX DOCUMENTATION. Each Party and any other recipient of payments under this Agreement shall provide to the
other Party, at the time or times reasonably requested by such other Parties or as required by applicable Law, such properly completed and duly executed documentation as will permit payments made under this Agreement to be made without, or at a
reduced rate of, withholding for taxes, and the applicable payment shall be made without (or at a reduced rate of) withholding to the extent permitted by such documentation, as reasonably determined by the Paying Party. 

 

	 	4.	 REPRESENTATIONS AND WARRANTIES 

LICENSEE represents and warrants to UNIVERSITY as of the Effective Date as follows: 

 

	 	4.1	 LICENSEE is a corporation duly organized, validly existing and in good standing under the laws of the country
of Denmark and has all requisite corporate power and authority to own and operate the business in which it is now engaged or currently proposed to be engaged. LICENSEE is duly qualified to do business as a foreign corporation and is in good standing
in such countries, states or jurisdictions as is necessary to enable it to carry on its business or own its properties. 

  

	 	4.2	 There is no action, suit proceeding or investigation pending or, to LICENSEE’s knowledge, threatened
against LICENSEE. 

  

	 	4.3	 LICENSEE is in compliance with all United States federal, state and local environmental laws and there are no
conditions currently existing or contemplated which are likely to subject LICENSEE to damages, penalties, injunctive relief, removal costs, remedial costs or cleanup costs under any such laws or assertions thereof. 

  
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[*] = Certain confidential information contained in this document, marked by brackets, is omitted because it is both (i) not material and (ii) would be
competitively harmful if publicly disclosed. 

	 	4.4	 LICENSEE will in good faith consider Institutions’ interests in the Licensed Product prior to terminating
this Agreement under section 14.l (Termination) and will meet to discuss the reasons for such termination with Institutions if any Institution so requests. 

  

	 	5.	 COMMERCIAL DILIGENCE AND MILESTONES 

 

	 	5.1	 DILIGENCE. LICENSEE shall use commercially reasonable efforts to develop, manufacture, market and sell Licensed
Products and Licensed Processes in the Territory. 

  

	 	5.2	 REPORTS. LICENSEE agrees to submit annual reports that summarize its efforts to develop Licensed Products and
Licensed Processes and markets for Licensed Products and Licensed Processes. Such reports shall include reasonable assurances by LICENSEE of its intent to actively develop the Licensed Products and Licensed Processes. 

 

	 	5.3	 DILIGENCE MILESTONE. LICENSEE hereby confirms that the first patient has been dosed in a Phase 3 clinical trial
(NCT03491462) evaluating the effectiveness of LICENSEE’s investigational therapy Arimoclomol in the treatment of amyotrophic lateral sclerosis. 

  

	 	5.4	 DEVELOPMENT MILESTONES. The Parties agree to the following milestone payment. The following milestone payment
shall not be creditable towards any other monies UNIVERSITY is due from LICENSEE, including but not limited to: royalty payments, and royalty payments associated with a Sublicensee’s sale of any Licensed Product(s) or Licensed Process(es). Upon
receiving Regulatory Approval for the pharmaceutical product containing Arimoclomol for which the intended Indication is Amyotrophic Lateral Sclerosis by the FDA or European Medicines Agency or other similar regulatory body in which the Study
results have been used in support of such Regulatory Approval, LICENSEE shall pay UNIVERSITY a one-time amount of one million one hundred fifty thousand U.S. dollars (US $1,150,000). 

 

	 	6.	 TERM 

The term of this Agreement shall commence on the Effective Date and shall remain in effect until the date that is ten (10) years from the
first commercial sale of the Licensed Product or Licensed Process in such country (on a country-by-country basis, the “Term”), unless terminated earlier
in accordance with the terms and conditions of this Agreement. 
  

	 	7.	 COMPLIANCE WITH LAWS 

 

	 	7.1	 COMPLIANCE WITH APPLICABLE LAWS. LICENSEE shall at all times during the term of this Agreement and for so long
as it shall use the Technology comply with all laws that may control the import, export, manufacture, use and other commercial exploitation of the Technology or any other activity undertaken pursuant to this Agreement. 

 

	 	7.2	 GOVERNMENT RIGHTS. LICENSEE understands that the Technology may have been developed under a funding agreement
with the United States Government and, if so, that the Government may have certain rights relative thereto. This Agreement is explicitly made subject to the Government’s rights under any agreement, and any applicable law or regulation. If there
is a conflict between an agreement, applicable law or regulation and this Agreement, the terms of the Government agreement, applicable law or regulation shall prevail. Specifically, this Agreement is subject to all of the terms and conditions of
Title 35 United States Code Sections 200 through 212 (to the extent applicable), including an obligation that Licensed Product(s) or Licensed Process(es) sold or produced in the United States be “manufactured substantially in the United
States,” and LICENSEE agrees to take all reasonable action necessary on its part as licensee to enable UNIVERSITY to satisfy its respective obligations thereunder, relating to the Technology. 

  
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[*] = Certain confidential information contained in this document, marked by brackets, is omitted because it is both (i) not material and (ii) would be
competitively harmful if publicly disclosed. 

	 	7.3	 EXPORT CONTROL REGULATIONS. It is understood that UNIVERSITY and LICENSEE are subject to United States laws and
regulations controlling the export of technical data, computer software, laboratory prototypes and other commodities (including the Arms Export Control Act, as amended and the Export Administration Act of 1979), and that its obligations hereunder
are contingent on compliance with applicable United States export laws and regulations. The transfer of certain technical data and commodities may require a license from the cognizant agency of the United States Government and/or written assurances
by LICENSEE that LICENSEE shall not export data or commodities to certain foreign countries without prior approval of such agency. UNIVERSITY neither represents that a license shall or shall not be required nor that, if required, it shall be issued.
LICENSEE represents and warrants that it will comply with, and will cause its Sublicensees and Affiliates to comply with all United States export control laws, rules and regulations. LICENSEE is solely responsible for any violation of such laws and
regulations by itself or its Affiliates or Sublicensees, and it will indemnify, defend and hold UNIVERSITY harmless for the consequences of any such violation. 

 

	 	8.	 INDEMNIFICATION AND LIMITATION OF LIABILITY 

 

	 	8.1	 INDEMNIFICATION 

  

	 	a.	 LICENSEE will defend, indemnify and hold harmless the Institutions, Institutions Affiliates, and their
respective trustees, officers, faculty, employees and students (the “Institutions’ Indemnitees”) against any and all losses, expenses, claims, actions, lawsuits and judgments thereon (including attorney’s fees through the
appellate levels) (collectively “ Liabilities”) which may be brought against Institutions’ Indemnities by third parties as a result of or arising out of: (i) any theory of product liability (including, but not limited to, actions
in the form of contract, tort, warranty, or strict liability ) concerning any product, process, or service made, used, sold or performed pursuant to any right or license granted under this Agreement; (ii) LICENSEE’s breach of this
Agreement; or (iii) the use, production, manufacture, sale, lease, consumption or advertisement by LICENSEE, its Sublicensees or Affiliates or its or their agents or employees of any Licensed Products or Licensed Processes; provided however,
LICENSEE shall not defend, indemnify or hold harmless any Institutions’ Indemnitee from any Liabilities to the extent that such Liabilities are finally determined to have resulted from the gross negligence or intentional misconduct of any
Institution or Institution Indemnitee. 

  

	 	b.	 LICENSEE will defend, indemnify and hold harmless the Institutions’ Indemnities against any and all
judgments and damages arising from any and all third party claims of infringement that may be asserted against any Institution Indemnities because of the manufacture, use, promotion and sale of Licensed Products or Licensed Processes. LICENSEE will
bear all costs and expenses incurred in connection with the defense of any such claims or as a result of any settlement made or judgment rendered on the basis of such claims. LICENSEE agrees to provide attorneys which shall be approved by
Institutions’ Indemnities at their sole and absolute discretion to defend against any actions brought or filed against any Institution Indemnitee hereunder with respect to the subject of indemnity contained herein, whether or not such actions
are rightfully brought; provided however, that any Institution Indemnitee shall have the right to retain its own counsel, at the reasonable expense of LICENSEE, if representation of such Institution Indemnitee by counsel retained by LICENSEE would
be inappropriate because of conflict of interests or otherwise. LICENSEE agrees to keep Institutions informed of the progress in the defense and disposition of such claim, and to consult with Institutions prior to any proposed settlement.

  
 Page 9 of 20 

[*] = Certain confidential information contained in this document, marked by brackets, is omitted because it is both (i) not material and (ii) would be
competitively harmful if publicly disclosed. 

	 	8.2	 LIMITATION OF LIABILITY. 

 

	 	a.	 Institutions shall have no responsibility with respect to LICENSEE’s own trademarks and trade name, and
LICENSEE in respect to the use thereof will defend, indemnify and hold harmless Institutions against any and all third party claims. 

  

	 	b.	 NOTWITHSTANDING ANYTHING TO THE CONTRARY IN THIS AGREEMENT, NEITHER PARTY SHALL BE LIABLE FOR ANY SPECIAL, LOST
PROFIT, EXPECTATION, INCIDENTAL, CONSEQUENTIAL, PUNITIVE, EXEMPLARY OR OTHER INDIRECT DAMAGES IN CONNECTION WITH ANY CLAIM ARISING OUT OF OR RELATED TO THIS AGREEMENT, WHETHER GROUNDED IN TORT (INCLUDING NEGLIGENCE), STRICT LIABILITY, CONTRACT, OR
OTHERWISE. UNIVERSITY’s TOTAL LIABILITY FOR ANY AND ALL CLAIMS OR ACTIONS ARISING FROM OR RELATED TO THIS AGREEMENT WILL IN NO EVENT EXCEED THE TOTAL AMOUNT PAID BY LICENSEE TO UNIVERSITY, BY AND ON BEHALF OF ITSELF AND THE INSTITUTIONS.

  

	 	8.3	 DISCLAIMER OF WARRANTIES. EACH OF THE INSTITUTIONS MAKE NO WARRANTIES, EXPRESS OR IMPLIED, AND HEREBY DISCLAIMS
ALL SUCH WARRANTIES, AS TO ANY MATTER WHATSOEVER, INCLUDING, WITHOUT LIMITATION, THE CONDITION OF ANY TECHNOLOGY, KNOW-HOW, LICENSED PRODUCT OR LICENSED PROCESS, WHETHER TANGIBLE OR INTANGIBLE, LICENSED UNDER
THIS AGREEMENT; OR OF MERCHANTABILITY, OR FITNESS FOR A PARTICULAR PURPOSE, OF SUCH TECHNOLOGY, KNOW-HOW, LICENSED PRODUCT OR LICENSED PROCESS. EACH OF THE INSTITUTIONS PROVIDES LICENSEE THE RIGHTS GRANTED
UNDER THIS AGREEMENT AS IS AND WITH ALL FAULTS, AND MAKES NO WARRANTY OR REPRESENTATION (A) REGARDING THE VALIDITY OR SCOPE OF THE TECHNOLOGY OR KNOW-HOW; OR (B) THAT EXPLOITATION OF THE TECHNOLOGY
OR KNOW-HOW WILL NOT INFRINGE ANY PATENTS OR OTHER INTELLECTUAL PROPERTY RIGHTS OF A THIRD PARTY. 

  

	 	8.4	 SURVIVAL. The provisions of this Section 8 shall survive the expiration or termination of this Agreement
for any reason. 

  

	 	9.	 REPORTS AND RECORDS 

 

	 	9.1	 By [*] of each year, LICENSEE shall furnish to UNIVERSITY a written annual report covering the preceding
calendar year. The report shall include information sufficient to enable UNIVERSITY to satisfy reporting requirements of the Government and for UNIVERSITY to ascertain progress by LICENSEE toward meeting the Agreement’s diligence requirements.
Each report shall describe, where relevant: LICENSEE’s progress toward commercialization of Licensed Product(s) and/or Licensed Process(es), including work completed, key scientific discoveries, summary of work-in-progress, current schedule of anticipated events or milestones, market plans for introduction of the Licensed Product(s) or Licensed Process(es), and significant corporate transactions involving the
Licensed Product(s) or Licensed Process(es). LICENSEE shall notify each Institution’s Office of Technology Transfer prior to commencing any clinical trials at Institution. In addition, the report shall specify (a) the number or amount of
Licensed Product(s) and Licensed Process(es) sold hereunder by LICENSEE and/or its Affiliates or Sublicensees, if any; (b) the total billings for all Licensed Product(s) and Licensed Process(es) sold, if any; (c) deductions as applicable;
(d) total royalties due, and (e) the names and addresses of all Sublicensees, if any. 

  

	 	9.2	 For a period of [*] years from the date of each report pursuant to Section 9.1, LICENSEE shall keep
records adequate to verify each such report and accompanying payment made to the UN IVERSITY under this Agreement, and an independent Certified Public Accountant or Accounting Finn selected by UNIVERSITY and acceptable to LICENSEE (the
“Independent Accountant”) 

  
 Page 10 of 20 

[*] = Certain confidential information contained in this document, marked by brackets, is omitted because it is both (i) not material and (ii) would be
competitively harmful if publicly disclosed. 

	 	
may have access, on reasonable notice during regular business hours, not to exceed twice per year, to such records to verify such reports and payments. LICENSEE’s acceptance of
UNNERSITY’s selection of said Independent Accountant shall not be unreasonably withheld. Such Independent Accountant shall not disclose to UNIVERSITY any information other than that information relating solely to the accuracy of, or necessity
for, the reports and payments made hereunder. The fees and expense of the Independent Accountant performing such verification shall be borne by UNIVERSITY, unless the audit reveals an underpayment of royalty by more than [*], in which case the cost
of the audit shall be paid by LICENSEE. 

  

	 	10.	 STANDARDS 

LICENSEE shall act in good faith to maintain satisfactory standards in respect to the nature of the Licensed Product or Licensed Process
manufactured and/or sold by LICENSEE. LICENSEE shall act in good faith to ensure that all Licensed Products or Licensed Processes manufactured and/or sold by it shall be of a quality which is appropriate to products or processes of the type here
involved. LICENSEE agrees that similar provisions shall be included by Sublicenses of all tiers. 
  

	 	11.	 ASSIGNMENT 

  

	 	11.1	 PERMITTED ASSIGNMENT. LICENSEE may assign or delegate its rights or obligations under this Agreement only under
the following circumstances: 

  

	 	a.	 by providing UNIVERSITY with written notice of the proposed assignment, including the proposed assignee’s
contact information, at least [*] days prior to the date of assignment, and obtaining UNIVERSITY’s express written consent to the proposed assignment, which consent shall not be unreasonably withheld; or 

 

	 	b.	 as part of a sale or change of control, regardless of whether such a sale or change of control occurs by
operation of law or through an asset sale, stock sale, merger or other combination, or any other transfer of: (i) LICENSEE’s entire business; or (ii) that part of LICENSEE’s business that exercises all rights granted under this
Agreement. 

  

	 	11.2	 CONDITIONS OF ASSIGNMENT. Prior to any assignment (including an assignment by operation of law), the proposed
assignee must agree in writing to UNIVERSITY to be bound by this Agreement. 

  

	 	11.3	 ANY OTHER ASSIGNMENT BY LICENSEE. Any attempt by LICENSEE to assign this Agreement that fails to comply with
Section 11.1 and 11.2 is null and void. 

  

	 	11.4	 SUCCESSORS AND ASSIGNS. This Agreement shall extend to and be binding upon the successors and legal
representatives and permitted assigns of UNIVERSITY and LICENSEE. 

  

	 	12.	 CONFIDENTIALITY. 

 

	 	12.1	 CONFIDENTIAL INFORMATION. From time to time during the term of this Agreement, any Institution (the
“Disclosing Party”) may disclose or make available to the LICENSEE (the “Receiving Party”) information about its business affairs, confidential intellectual property, trade secrets,
know-how, copyrights, trademarks, designs, data, algorithms, code, and oral communications relating to the Technology, third party confidential information and other sensitive or proprietary information
(collectively, “Confidential Information”). Confidential Information shall not include information that, at the time of disclosure and as established by documentary evidence: 

 

	 	a.	 is or becomes generally available to and known by the public other than as a result of, directly or indirectly,
any breach of this Section 12 by the Receiving Party or any of its employees, agents or representatives; 

  
 Page 11 of 20 

[*] = Certain confidential information contained in this document, marked by brackets, is omitted because it is both (i) not material and (ii) would be
competitively harmful if publicly disclosed. 

	 	b.	 is or becomes available to the Receiving Party on a non-confidential
basis from a third party source, provided that such third party is not and was not prohibited from disclosing such Confidential Information; 

  

	 	c.	 was known by or in the possession of the Receiving Party or its employees, agents or representatives prior to
being disclosed by or on behalf of the Disclosing Party; or 

  

	 	d.	 was or is independently developed by the Receiving Party without reference to or use of, in whole or in part,
any of the Disclosing Party’s Confidential Information. 

  

	 	12.2	 RECEIVING PARTY OBLIGATIONS. The Receiving Party shall: 

 

	 	a.	 protect and safeguard the confidentiality of the Disclosing Party’s Confidential Information with at least
the same degree of care as the Receiving Party would protect its own Confidential Information, but in no event with less than a commercially reasonable degree of care; 

 

	 	b.	 not use the Disclosing Party’s Confidential Information, or permit it to be accessed or used, for any
purpose other than to exercise its rights or perform its obligations under this Agreement; and 

  

	 	c.	 not disclose any such Confidential Information to any person or entity, except to the Receiving Party’s
employees, agents or representatives who need to know the Confidential Information to assist the Receiving Party, or act on its behalf, to exercise its rights or perform its obligations under this Agreement and who are bound by written obligations
of confidentiality and restrictions on use that cover such Confidential Information and are at least as stringent as those set forth in this Agreement. 

  

	 	12.3	 COURT OR GOVERNMENT ORDER. Notwithstanding anything in this Agreement to the contrary, Receiving Party may make
disclosures of Confidential Information of the Disclosing Party to the extent required to be disclosed pursuant to applicable federal, state or local law or a valid order issued by a court or governmental agency of competent jurisdiction; provided,
that (a) the Receiving Party gives the Disclosing Party prompt written notice of such requirement prior to disclosure, (b) the Receiving Party reasonably cooperates with the Disclosing Party’s efforts to limit the scope of the
information to be provided or to obtain an order protecting the information from public disclosure, and (c) the Receiving Party discloses only that portion of the Confidential Information that is legally required to be disclosed.

  

	 	12.4	 RETURN OF CONFIDENTIAL INFORMATION. Upon expiration or termination of the Agreement, at the Disclosing
Party’s written request, the Receiving Party and its employees, agents and representatives shall promptly return to the Disclosing Party all copies, whether in written, electronic or other form or media, of the Disclosing Party’s
Confidential Information, or destroy all such copies and certify in writing to the Disclosing Party that such Confidential Information has been destroyed; provided, however, that the Receiving Party may retain (i) such documents and records to
the extent required by any law, rule, or regulation or its document retention policy and (ii) materials stored electronically in a back-up archival computer system only to the extent it is not practical
to destroy the same; provided, further, that such information retained pursuant to the preceding proviso shall remain subject to the confidentiality obligations of this Agreement notwithstanding any termination or expiration hereof.

  
 Page 12 of 20 

[*] = Certain confidential information contained in this document, marked by brackets, is omitted because it is both (i) not material and (ii) would be
competitively harmful if publicly disclosed. 

	 	12.5	 REMEDIES. The Receiving Party shall be responsible for any breach of this Section 12 caused by any of its
employees, agents or representatives. The Disclosing Party may seek equitable relief (including injunctive relief) against the Receiving Party to prevent the breach or threatened breach of this Section 12.5 and to secure its enforcement, in
addition to all other remedies available at law. 

  

	 	12.6	 SURVIVAL. The provisions of this Section 12 shall survive the expiration or termination of this Agreement
for [*] years. 

 13. NOTICE. Any notice, payment, report or other correspondence (hereinafter collectively referred
to as “correspondence”) required or permitted to be given hereunder shall be mailed by certified mail or delivered by hand to the Party to whom such correspondence is required or permitted to be given hereunder. If mailed, any such notice
shall be deemed to have been given when mailed as evidenced by the postmark at point of mailing. If delivered by hand, any such correspondence shall be deemed to have been given when received by the Party to whom such correspondence is given, as
evidenced by written and dated receipt of the receiving Party. 
 All correspondence to LICENSEE shall be addressed as follows: 

Chief Executive Officer 

Orphazyme ApS 
 Ole Maaloes Vej
3 
 DK-2200 Copenhagen N 

Denmark 
 WITH A COPY TO: 

Dechert LLP 
 Attention: David
E. Schulman 
 1900 K Street NW 

Washington, DC 20006 
 All
correspondence to UNIVERSITY shall be addressed, in duplicate, as follows: 
 FOR NOTICE: 

Assistant Vice President 

Financial Operations 

University of Miami 
 1320 South
Dixie Highway, Suite 1250 
 Gables One Tower 

Coral Gables, FL 33146 
 WITH A
COPY TO: 
 Office of the General Counsel 

University of Miami 
 1320 South
Dixie Highway, Suite 1250 
 Gables One Tower 

Coral Gables, FL 33146 
 FOR
NOTICE AND PAYMENT: 
 Office of Technology Transfer 

University of Miami 
 1951 NW 7th Avenue, Suite 300 
 Miami, FL 33136 

Either Party may change the address to which correspondence to it is to be addressed by notification as provided herein. 

  
 Page 13 of 20 

[*] = Certain confidential information contained in this document, marked by brackets, is omitted because it is both (i) not material and (ii) would be
competitively harmful if publicly disclosed. 

	 	14.	 MISCELLANEOUS PROVISIONS 

 

	 	14.1	 TERMINATION. 

  

	 	a.	 LICENSEE shall have the right to terminate this Agreement upon [*] prior written notice to UNIVERSITY, during
which time LICENSSEE shall pay any amounts due and outstanding to UNIVERSITY. 

  

	 	b.	 UNIVERSITY and LICENSEE shall have the right to terminate this Agreement if the other Party commits a material
breach of an obligation under this Agreement and fails to cure any such breach within [*] of receipt of written notice from the non-breaching Party. A material breach shall include but not be limited to the
following: (i) failure to deliver to UNIVERSITY any payment at the time such payment is due under this Agreement, which payment is not the subject of a good faith dispute, (ii) failure to meet or achieve milestone schedule,
(iii) failure to possess and maintain required insurance coverage in any material respects, or (iv) knowing delivery of a false report to UNIVERSITY. Such termination shall be effective upon further written notice to the breaching Party
after failure by the breaching Party to cure. 

  

	 	c.	 If the UNIVERSITY commits a material breach or defaults, then LICENSEE has no duty to continue the payment of
royalties and other consideration as set forth in Article 3 and Section 5.4 of this Agreement. 

  

	 	d.	 The license and rights granted in this Agreement have been granted on the basis of the special capability of
LICENSEE to perform research and development work leading to the manufacture and marketing of the Licensed Product(s) or Licensed Process(es). Accordingly, LICENSEE covenants and agrees that in the event any proceedings under Title 11, United States
Code or any amendment thereto, be commenced by or against LICENSEE, and, if against LICENSEE, said proceedings shall not be dismissed with prejudice before either an adjudication in bankruptcy or the confirmation of a composition, arrangement, or
plan of reorganization, or in the event LICENSEE shall be adjudged insolvent or make an assignment for the benefit of its creditors, or if a writ of attachment or execution be levied upon the license hereby created and not be released or satisfied
within [*] thereafter, or if a receiver be appointed in any proceeding or action to which LICENSEE is a party with authority to exercise any of the rights or privileges granted hereunder and such receiver be so discharged within a period of [*]
after his appointment, any such event shall be deemed to constitute a breach of this Agreement by LICENSEE and UNIVERSITY, at the election of UNIVERSITY, but not otherwise, ipso facto, and without notice or other action by UNIVERSITY, shall
terminate this Agreement and all rights of LICENSEE hereunder and all rights of any and all persons claiming under LICENSEE. 

  

	 	e.	 LICENSEE agrees that breach of terms of this Agreement would immediately and irreparably damage UNIVERSITY in a
way not capable of being fully compensated by monetary damages and accordingly, UNIVERSITY is entitled to seek injunctive relief in addition to such other relief to which it may be entitled at law or in equity. 

 

	 	f.	 Upon termination of this Agreement for any reason, LICENSEE shall promptly pay to UNIVERSITY any amounts
accrued as of the effective date of such termination. Any termination of this Agreement shall be without prejudice to UNIVERSITY’s right to recover all amounts accruing to UNIVERSITY prior to such the effective date termination and
cancellation. Except as otherwise provided, should this Agreement be terminated for any reason, LICENSEE shall have no rights, express or implied, under any intellectual property rights which are the subject matter of this Agreement including
Technology, nor have the right to recover any royalties paid to UNIVERSITY hereunder. Upon termination, LICENSEE shall have the right to dispose of Licensed Products then in their possession

  
 Page 14 of 20 

[*] = Certain confidential information contained in this document, marked by brackets, is omitted because it is both (i) not material and (ii) would be
competitively harmful if publicly disclosed. 

	 	
and to complete existing contracts for such Licensed Products, so long as contracts are completed within [*] from the date of termination, subject to the payment of royalties to UNIVERSITY as
provided in Section 3 hereof; it being understood and agreed, following the termination of this Agreement for any reason and completion of existing contracts for such Licensed Products as described in this sentence, LICENSEE shall have no
further payment obligations (other than those accrued prior to the termination) to UNIVERSITY under this Agreement. LICENSEE agrees to destroy progeny and derivatives thereof remaining in LICENSEE’s possession after [*] from the date of
termination. Failure to terminate on any basis shall not prejudice or impact the UNIVERSITY’s rights and ability to subsequently terminate for the same or a related basis. 

 

	 	g.	 Notwithstanding anything to the contrary, UNIVERSITY, by and on behalf of itself and each of the Institutions,
hereby grants to LICENSEE and LICENSEE hereby accepts a non-exclusive, royalty-free, fully paid-up, non-terminable, sublicensable
(through multiple tiers) license in the Territory for the Field of Use, to use the safety data generated under the Study to research, develop, make, have made, use, sell and import any pharmaceutical product (including the Licensed Product).

  

	 	14.2	 INSURANCE. 

  

	 	a.	 Prior to the commencement of clinical trials, LICENSEE must maintain commercial general liability insurance in
the amounts of not less than [*] per incident and [*] annual aggregate. After the commencement of the first clinical trial for the first Licensed Product or Licensed Process but prior to the first commercial sale of a Licensed Product or Licensed
Process, LICENSEE must maintain commercial general liability insurance of not less than [*] per incident and clinical trials liability insurance of not less than [*]. After the first commercial sale of a Licensed Product or Licensed Process,
LICENSEE must maintain commercial general liability insurance in the amounts of not less than [*] per incident and [*] annual aggregate. Immediately prior to the commencement of the first clinical trial for the first Licensed Product or Licensed
Process, [*]. After the first commercial sale of a Licensed Product or Licensed Process, LICENSEE shall maintain products liability/completed operations and clinical trials insurance coverage in the amount of [*]. 

 

	 	b.	 LICENSEE shall not cancel such insurance without [*] prior notice to UNIVERSITY. Such cancellation shall be
cause for termination. 

  

	 	c.	 The terms of this Section 14.2 shall survive termination of this Agreement. 

 

	 	14.3	 USE OF NAME. LICENSEE shall not, without the prior written consent and approval of the respective Institution,
use any name, trade name, trademark, or other designation of such Institution, or any of their Affiliates, trustees, faculty, students, employees or departments, or any adaptation thereof (including contraction, abbreviation or simulation) in any
publication, including advertising, promotional or sales literature, or any other activities or context. 

  

	 	14.4	 GOVERNING LAW; VENUE AND DISPUTE RESOLUTION. 

 

	 	a.	 This Agreement shall be considered as having been entered into in [*], and shall be construed and interpreted
in accordance with the laws of [*]. In any action or proceeding arising out of or relating to this Agreement (an “Action”), each of the Parties hereby irrevocably submits to the jurisdiction of [*], and further agrees that any
Action shall be heard and determined in such [*] court. Each Party hereby irrevocably waives, to the fullest extent it may effectively do so, the defense of an inconvenient forum to the maintenance of any Action in [*]. 

  
 Page 15 of 20 

[*] = Certain confidential information contained in this document, marked by brackets, is omitted because it is both (i) not material and (ii) would be
competitively harmful if publicly disclosed. 

	 	b.	 If a dispute arises between the Parties relating to the interpretation or performance of this Agreement or the
grounds for the termination thereof, the Parties agree to hold a meeting, attended by individuals with decision-making authority regarding the dispute, to attempt in good faith to negotiate a resolution of the dispute prior to pursuing other
available remedies. If the dispute remains unresolved [*] after the first meeting for the purpose of dispute resolution, then each Party shall have the right to pursue other remedies legally available to resolve the dispute. 

 

	 	14.5	 CAPTIONS. The captions and section headings of this Agreement are solely for the convenience of reference and
shall not affect its interpretation. 

  

	 	14.6	 SEVERABILITY. Should any part or provision of this Agreement be held unenforceable or in conflict with the
applicable laws or regulations of any jurisdiction, the invalid or unenforceable part or provision shall be replaced with a provision which accomplishes, to the extent possible, the original business purpose of such part or provision in valid and
enforceable manner, and the remainder of this Agreement shall remain binding upon the Parties hereto. 

  

	 	14.7	 COUNTERPARTS. This Agreement may be executed in counterparts, each of which shall be deemed an original, but
all of which together shall constitute one and the same instrument. 

  

	 	14.8	 SURVIVAL. 

  

	 	a.	 The provisions of Sections 1 (Definitions), 8 (Indemnification and Limitation of Liability), 11 (Assignment),
12 (Confidentiality), 13 (Notice) and 14 (Miscellaneous Provisions), shall survive the termination or expiration of this Agreement and shall remain in full force and effect. 

 

	 	b.	 The provisions of this Agreement which do not survive termination or expiration hereof (as the case may be)
shall, nonetheless, be controlling on, and shall be used in construing and interpreting, the rights and obligations of the Parties hereto with regard to any dispute, controversy or claim which may arise under, out of, in connection with, or relating
to this Agreement. 

  

	 	c.	 Sublicenses in good standing shall survive termination of this license as a direct license from UNIVERSITY,
provided that Sublicensees assume the obligations set forth in the definitive agreement. UNIVERSITY will enter into a direct agreement with such Sublicensees upon LICENSEE’s written request. 

 

	 	14.9	 AMENDMENT. No amendment or modification of the terms of this Agreement shall be binding on either Party unless
reduced to writing and signed by an authorized officer of each Party. 

  

	 	14.10	 NON-WAIVER. No failure or delay on the part of a Party in exercising
any right hereunder will operate as a waiver of, or impair, any such right. No waiver of any of the provisions of this Agreement shall be effective unless it is in writing, and signed by the Party against whom it is asserted, and any such written
waiver shall only be applicable to the specific instance to which it relates and shall not be deemed to be a continuing or future waiver. No single or partial exercise of any such right will preclude any other or further exercise thereof or the
exercise of any other right. No waiver of any such right will be deemed a waiver of any other right hereunder. 

  

	 	14.11	 INDEPENDENT CONTRACTOR RELATIONSHIP. This Agreement is not intended to create nor shall be construed to create
any relationship between LICENSEE and UNIVERSITY other than that of independent entities contracting for the purpose of effecting provisions of this Agreement. It is further expressly agreed that no work, act, commission or omission of any Party,
its agents, servants or employees, pursuant to the terms and conditions of this Agreement, shall be construed to make or render any Party, its agents, servants or employees, an agent, servant, representative, or employee of, or joint venturer with,
the other Party. Neither Party shall have any right to bind or obligate the other Party in any way nor shall it represent that it has any right to do so. 

  
 Page 16 of 20 

[*] = Certain confidential information contained in this document, marked by brackets, is omitted because it is both (i) not material and (ii) would be
competitively harmful if publicly disclosed. 

	 	14.12	 REPRESENTATION BY COUNSEL. Each Party acknowledges that it has had the opportunity to be represented by counsel
of such Party’s choice with respect to this Agreement. In view of the foregoing and notwithstanding any otherwise applicable principles of construction or interpretation, this Agreement shall be deemed to have been drafted jointly by the
Parties and in the event of any ambiguity, shall not be construed or interpreted against the drafting Party. 

  

	 	14.13	 NO THIRD PARTY BENEFICIARIES. No third persons or entities are intended to be or are third party beneficiaries
of or under this Agreement, including, without limitation, Sublicensees and Affiliates. Nothing in this Agreement shall be construed to create any liability on the part of the Parties or their respective directors, officers, shareholders, employees
or agents, as the case may be, to any such third parties for any act or failure to act of any Party hereto. 

  

	 	14.14	 CONFLICTS. LICENSEE understands and agrees that Institutions ‘ personnel who are engaged by LICENSEE,
whether as consultants, employees or otherwise, or who possess a material financial interest in LICENSEE, are subject to Institutions’ rules regarding outside activities and financial interests set forth in each Institutions respective
Intellectual Property Policy. Any term or condition of an agreement between LICENSEE and such Institution’s personnel which seeks to vary or override such personnel’s obligations to Institution may not be enforced against such personnel or
Institution without the express written consent of an individual authorized to vary or waive such obligations on behalf of Institution. Furthermore, should an interest of LICENSEE conflict with the interest of any Institution, Institutions’
personnel are obligated to resolve such conflicts according to the guidelines and policies set forth by the respective Institution. 

  

	 	14.15	 ENTIRE AGREEMENT. This Agreement constitutes the entire agreement between the Parties hereto respecting the
subject matter hereof, and supersedes and terminates all prior agreements respecting the subject matter hereof, whether written or oral, and may be amended only by an instrument in writing executed by both Parties hereto. UNIVERSITY hereby confirms
that each INSTITUTION has granted to UNIVERSITY an exclusive right to negotiate, execute, license and administer its rights in the Technology and Know-How pursuant to the
Three-Way Inter-Institutional Agreement. 

  

	 	14.16	 FORCE MAJEURE. Neither Party shall be held responsible for any delay of failure in performance of any part of
this Agreement to the extent such delay or failure is caused by fire, flood, explosion, war, embargo, government requirement, civil or military authority, act of God, or other similar causes beyond its control and without the fault or negligence of
the delayed or non-performing Party. The affected Party will notify the other Party in writing within ten (10) days after the beginning of any such cause that would affect its performance.
Notwithstanding, if a Party’s performance is delayed for a period exceeding [*] from the date the other Party receives notice under this paragraph, the non-affected Party will have the right, without any
liability to the other Party, to terminate this agreement. 

  

	 	14.17	 TAX-EXEMPT STATUS. LICENSEE acknowledges that Institutions’ hold
the status of an exempt organization under the Internal Revenue Code of 1986, as amended. LICENSEE also acknowledges that certain facilities in which the Technology was developed may have been financed through offerings of tax-exempt bonds. If the Internal Revenue Service determines, or if Institutions’ reasonably determine, that any term of this Agreement jeopardizes the tax-exempt status
of Institutions or the bonds used to finance Institutions’ facilities, the relevant term is invalid and the Parties shall modify the term accordingly. 

{Signature Page Follows.} 

  
 Page 17 of 20 

[*] = Certain confidential information contained in this document, marked by brackets, is omitted because it is both (i) not material and (ii) would be
competitively harmful if publicly disclosed. 

 IN WITNESS WHEREOF, the Parties hereto have caused this Agreement to be executed by their respective
officers thereunto duly authorized to be effective as of the Effective Date. 
  

					
	LICENSEE	 		  	UNIVERSITY on behalf of itself, EMORY and MGH
			
	 /s/ Anders Fink Vadsholt
	 		  	 /s/ Norma Sue Kenyon, Ph.D.

	Signature	 		  	Signature
			
	 Anders Fink Vadsholt
	 		  	 Norma Sue Kenyon, Ph.D.

	Printed Name	 		  	Printed Name
			
	 Chief Financial Officer
	 		  	 Vice Provost for Innovation

	Printed Title	 		  	Printed Title

 {Signature Page to University of Miami and Orphazyme License Agreement} 

  
 [*] = Certain confidential information
contained in this document, marked by brackets, is omitted because it is both (i) not material and (ii) would be competitively harmful if publicly disclosed. 

 APPENDIX A 

TECHNOLOGIES/INTELLECTUAL PROPERTY 
 [*]

  
 [*] = Certain confidential information
contained in this document, marked by brackets, is omitted because it is both (i) not material and (ii) would be competitively harmful if publicly disclosed.EX-10.3

 Exhibit 10.3 

Execution Version 
 [*] = Certain
confidential information contained in this document, marked by brackets, is omitted because it is both (i) not material and (ii) would be competitively harmful if publicly disclosed. 

LICENSE AGREEMENT 

dated October 31 2017 

between 
 Orphazyme A/S

 and 
 KU Center for
Technology Commercialization, Inc. 
 and 

University of Kansas 
 and

 Kansas Life Sciences Development Company, Inc. 

and 
 UCL Business PLC 

 TABLE OF CONTENTS 

 

					
	ARTICLE 1. DEFINITIONS	  	 	1	 
	ARTICLE 2. LICENSE GRANT	  	 	4	 
	ARTICLE 3. TERM OF AGREEMENT	  	 	5	 
	ARTICLE 4. ROYALTIES	  	 	6	 
	ARTICLE 5. COMMERCIAL DILIGENCE & MILESTONES	  	 	6	 
	ARTICLE 6. EQUITY OWNERSHIP	  	 	7	 
	ARTICLE 7. CONFIDENTIALITY	  	 	8	 
	ARTICLE 8. QUARTERLY & ANNUAL REPORTS	  	 	8	 
	ARTICLE 9. PAYMENTS, RECORDS AND AUDITS	  	 	9	 
	ARTICLE 10. PATENT MARKING	  	 	10	 
	ARTICLE 11. PATENT PROSECUTION AND MAINTENANCE	  	 	10	 
	ARTICLE 12. TERMINATION BY LICENSOR	  	 	10	 
	ARTICLE 13. TERMINATION BY LICENSEE	  	 	11	 
	ARTICLE 14. DISPOSITION OF LICENSED PRODUCTS ON HAND	  	 	12	 
	ARTICLE 15. WARRANTY BY LICENSOR	  	 	12	 
	ARTICLE 16. PATENT ENFORCEMENT	  	 	13	 
	ARTICLE 17. INSURANCE	  	 	13	 
	ARTICLE 18. WAIVER	  	 	14	 
	ARTICLE 19. ASSIGNABILITY	  	 	14	 
	ARTICLE 20. INDEMNIFICATION BY LICENSEE	  	 	14	 
	ARTICLE 21. NOTICES	  	 	14	 
	ARTICLE 22. REGULATORY COMPLIANCE	  	 	15	 
	ARTICLE 23. GOVERNING LAW	  	 	16	 
	ARTICLE 24. RELATIONSHIP OF PARTIES	  	 	16	 
	ARTICLE 25. USE OF NAMES	  	 	16	 
	ARTICLE 26. DISPUTE RESOLUTION	  	 	16	 
	ARTICLE 27. GENERAL PROVISIONS	  	 	17	 

  
 [*] = Certain confidential information
contained in this document, marked by brackets, is omitted because it is both (i) not material and (ii) would be competitively harmful if publicly disclosed. 

 LICENSE AGREEMENT 

This License Agreement (“Agreement”) is entered into as of October 31, 2017 by and among the KU CENTER FOR TECHNOLOGY
COMMERCIALIZATION, INC. d/b/a KU Innovation and Collaboration, having its principal place of business at 2029 Becker Drive, Suite 142, Lawrence, KS 66047, hereinafter referred to as “KUCTC,” UNIVERSITY OF KANSAS, on behalf of University of
Kansas Medical Center, having its principal place of business at 3901 Rainbow Boulevard, Kansas City, Kansas 66160, hereinafter referred to as “KUMC,” KANSAS LIFE SCIENCES DEVELOPMENT COMPANY, INC., having its principal place of business
at 3901 Rainbow Boulevard, Kansas City, Kansas 66160, hereinafter referred to as “KLSDC,” and collectively, with KUCTC and KUMC, “Kansas,” UCL BUSINESS PLC, having its principal place of business at 97 Tottenham Court Road,
London W1T 4TP, UK, hereinafter referred to as “UCLB,” and ORPHAZYME A/S, having its principal place of business at Ole Maaløes Vej 3, DK-2200 N, Copenhagen, Denmark, hereinafter referred to
as “Licensee.” For purposes of this Agreement: (a) each of Kansas and UCLB will be referred to as the “Licensor” and (b) each of Kansas, UCLB and Licensee shall be referred to as a “Party” and collectively as
the “Parties.” 
 WITNESSETH 

WHEREAS, certain data and know-how have been generated in the course of research in connection with
the Study (as defined below) conducted by and on behalf of the University of Kansas (“KUMC”) and University College London (“UCL”); 

WHEREAS, KUMC and KUCTC have an agreement wherein KUCTC is the manager of intellectual property owned by KUMC; 

WHEREAS, UCL has assigned to UCLB all of its right, title and interest in and to any data and know-how
generated in the course of research in connection with the Study undertaken at UCL; 
 WHEREAS, each of the Licensors desires that certain
data and know-how generated in the Study be developed and utilized to the fullest extent so that their benefits can be enjoyed by the general public; 

WHEREAS, Licensee wishes to obtain from each of the Licensors a license to all of the data and
know-how generated in the Study for the commercial development, production, manufacture, use and sale of Licensed Products, and each of the Licensors is willing to grant such a license upon the terms and
conditions hereinafter set forth; 
 WHEREAS, certain data and know-how generated in the Study were
developed in the course of research sponsored in part by the U.S. Government, and as a consequence are subject to overriding obligations of Kansas to the U.S. Government; 

NOW THEREFORE, for and in consideration of the covenants, conditions and undertakings hereinafter set forth, the Parties hereby agree as
follows: 
 ARTICLE 1. DEFINITIONS 
  

	1.1	 “Affiliate” means any company or other business entity that, directly or indirectly, controls,
or is controlled by, or is under common control by Licensee. Solely for purposes of this definition, the term “control” means the possession of the power to direct or cause the direction of the management and policies of the entity,
whether through ownership of voting securities or by contract. Control will be presumed if an entity owns, either of record or beneficially, at least fifty percent (50%) of the voting stock of the other entity. An entity will be deemed an Affiliate
only while such ownership or control relationship continues. 

  

	1.2	 “Commercially Diligent Efforts” means, with respect to a Licensed Product, the diligent
exercise, dedication and expenditure of efforts, money, personnel, and resources as reasonably needed to develop, manufacture, market and sell the Licensed Product. Such efforts shall be documented and must be consistent with those utilized by
companies of similar size and type that have successfully developed products and services similar to the Licensed Product. At a minimum, Commercially Diligent Efforts shall be based upon the development plan, attached as Exhibit B and incorporated
by reference, submitted to each of the Licensors by Licensee as required under Article 5. In determining Commercially Diligent Efforts with respect to a particular Licensed Product, Licensee may not reduce such efforts due to the competitive,
regulatory or other impact of any other product or method that it owns, licenses or is developing or commercializing for the Intended Indication. 

  
 1 

[*] = Certain confidential information contained in this document, marked by brackets, is omitted because it is both (i) not material and (ii) would be
competitively harmful if publicly disclosed. 

	1.3	 “Effective Date” means the date first set forth above. 

 

	1.4	 “Entity” means a corporation, an association, a joint venture, a partnership, a trust, a
business, an institution, an individual, a government or political subdivision thereof, including an agency, or any other organization that can exercise independent legal standing. 

 

	1.5	 “Fair Market Value” means the cash consideration which Licensee or its Sublicensee would
realize from an unaffiliated, unrelated buyer in an arm’s length sale of an identical item sold in the same quantity, under the same terms, and at the same time and place. 

 

	1.6	 “FDA” means the United States Food and Drug Administration, or any successor agency thereof.

  

	1.7	 “Field of Use” means any use or purpose, including the treatment, palliation, diagnosis or
prevention of any human disease, disorder or condition. 

  

	1.8	 “Grant” means that certain Food and Drug Administration grant assigned grant number
1R01FD004809-01A2. 

  

	1.9	 “Insolvent” means being unable to meet one’s debt obligations to another Entity as such
debt obligations become due and not being able to provide reasonable financial assurances of becoming able to meet such obligations. 

  

	1.10	 “IND” means any Investigational New Drug application, filed with the FDA pursuant to Part 312
of Title 21 of the U.S. Code of Federal Regulations, including any supplements or amendments thereto. References herein to IND shall include, to the extent applicable, any comparable filing(s) outside the United States (such as a Clinical Trial
Application in the countries that are officially recognized as member states of the European Union). 

  

	1.11	 “Intended Indication” means the treatment, palliation, diagnosis or prevention in humans of
sporadic Inclusion Body Myositis. 

  

	1.12	 “Licensed Data” means in relation to each Licensor, any and all data (in anonymized form,
except as provided in Section 2.2) generated during the conduct and analysis of the Study (including but not limited to the trial master file and any Study reports) and any relevant data (in anonymized form, except as provided in
Section 2.2) from any prior and/or related preclinical and clinical investigational study in that Licensor’s possession (including without limitation as required for the purpose of filing a new drug application or marketing authorisation
application (or other comparable filings) to regulatory authorities for registration and market authorization and pricing and reimbursement approval (as applicable)) of arimoclomol in treatment of sIBM, and any inventions and other know-how included in any such data. 

  

	1.13	 “Licensed Product” means any pharmaceutical product containing arimoclomol as an active
ingredient, in all forms, presentations and formulations (including manner of delivery, dosage and combinations with other therapeutic agents). 

  

	1.14	 “Net Sales” means the gross revenue and other consideration paid or given to or received by or
on behalf of Licensee or its Affiliates or Sublicensees for Licensed Products for the Intended Indication which are sold, leased or otherwise commercialized by or for Licensee or any of its Affiliates or Sublicensees; however, sales or other
transfers of Licensed Products (a) between Licensee and any Sublicensee and its respective Affiliates (except where such Affiliates or Sublicensees are end users or consumers) or (b) in connection with any sale of the Licensee (including
any asset sale to any acquirer) shall in case of clauses (a) and/or (b) be excluded from the computation of Net Sales, and no payments will be payable to any Licensors on such sales or transfers, provided always that the royalties due
under this Agreement are paid to the Licensors on the subsequent sale of the Licensed Product by the relevant Affiliate or Sublicensee or acquirer; it being understood and agreed that “Net Sales” shall be reduced by the following
documented deductions directly attributable to the sale of such Licensed Product: 

  

	 	a.	 quantity and/or patient program discounts, retroactive price reductions, charge-back payments and rebates
actually granted to purchases of a Licensed Product; 

  
 2 

[*] = Certain confidential information contained in this document, marked by brackets, is omitted because it is both (i) not material and (ii) would be
competitively harmful if publicly disclosed. 

	 	b.	 allowances or credits to third parties for damaged goods, rejections or returns, including such Licensed
Product returned in connection with recalls or withdrawals; 

  

	 	c.	 taxes, regulatory fees (including but not limited to annual fees due under Section 9008 of the United
States Patient Protection and Affordable Care Act of 2010 (Pub. L. No. 111-48) and other comparable laws), tariffs and duties levied on, absorbed or otherwise imposed on the sales of a Licensed Product by
a governmental authority, including value-added taxes, or other governmental charges otherwise imposed upon the billed amount, to the extent borne by Licensee (or its Affiliates or Sublicensees); and 

 

	 	d.	 outbound transportation charges, including freight, postage, customs charges, shipping and insurance charges
for delivery of such Licensed Product, provided in each case that the amounts are separately charged to the purchaser on the relevant invoice. 

A Licensed Product shall be considered sold when it is shipped, delivered, or invoiced, whichever is earlier. No deductions shall be made from
Net Sales for commission paid to individuals whether they are with independent sales agencies or are regularly employed by Licensee or its Affiliates or Sublicensees and are on its or their payroll, or for the cost of collections. Subject to the
terms and conditions of this Agreement, in the event Licensee transfers a Licensed Product to a third party in a bona fide arm’s length transaction, for consideration, in whole or in part, other than cash, then the Net Sales price for such
Licensed Product shall be deemed to be the standard invoice price then being invoiced by Licensee in an arm’s length transaction with similar companies and in the absence of such standard invoice price, then the reasonable Fair Market Value of
the Licensed Product. Components of Net Sales shall be determined in the ordinary course of business using the accrual method of accounting in accordance with generally accepted accounting practices. 

If Licensee or any Affiliate or Sublicensee sells, leases or otherwise commercializes any Licensed Product at a reduced fee or price for the
purpose of promoting other products, goods or services or for the purpose of facilitating the sale, license or lease of other products, goods or services, then Licensee shall pay to the applicable Licensors a royalty under Article 4 based upon the
Fair Market Value of the License Product. 
  

	1.15	 “Patent Rights” means, in respect of each Licensor, and solely with respect to inventions that
were discovered, generated, invented or improved by or on behalf of that Licensor or its Affiliates (including without limitation any and all employees any Licensor or its Affiliates) during the conduct and analysis of the Study, all rights, title
and interests in and to (a) all national, regional and international patents and patent applications filed in any country of the world including provisional patent applications and all supplementary protection certificates, (b) all patent
applications filed either from such patents, patent applications or provisional applications or from an application claiming priority from any of these, including any continuation, continuation-in part,
divisional, provisional, converted provisionals and continued prosecution applications, or any substitute applications, (c) any patent issued with respect to or in the future issued from any such patent applications, including utility models,
petty patents and design patents and certificates of invention, and (d) any and all extensions or restorations by existing or future extension or restoration mechanisms, including revalidations, reissues, reexaminations and extensions
(including any supplementary protection certificates and the like) of the foregoing patents or patent applications; it being understood and agreed that “Patent Rights” shall be limited to all of the foregoing as and to the extent generated
during the conduct and analysis of the Study. 

  

	1.16	 “Study” means the clinical trial for testing arimoclomol in sporadic Inclusion Body Myositis
(“sIBM”) that is the subject of the Grant. 

  

	1.17	 “Sublicensee” means any party other than an Affiliate which enters into an agreement or
arrangement with Licensee and receives a license grant from Licensee to use the Licensed Data and/ or Patent Rights to manufacture, have manufactured, offer for sale, sell, lease, use, practice, and/or import the Licensed Product for the Intended
Indication, subject to the then-current applicable article, item, service, technology, and technical data-specific requirements of the U.S. export laws and regulations. 

 

	1.18	 “Territory” means worldwide. 

  
 3 

[*] = Certain confidential information contained in this document, marked by brackets, is omitted because it is both (i) not material and (ii) would be
competitively harmful if publicly disclosed. 

 ARTICLE 2. LICENSE GRANT 

 

	2.1	 Exclusive License to Licensed Data, and Patent Rights 

Subject to the terms and conditions set forth herein, each Licensor hereby grants to Licensee, in consideration of the royalties payable under
Article 4 and issuance of the Shares pursuant to and in accordance with Article 6, a royalty-bearing exclusive license of its rights in and to and under the Licensed Data, and Patent Rights to research, develop, make, have made, use, offer for sale,
sell, import and/or otherwise commercialize the Licensed Product in the Field of Use throughout the Territory. This grant is subject to any rights of the Government of the United States as set forth in Section 2.3. Licensee covenants and agrees
to [*] within [*] following the Effective Date [*] in connection with [*] this Agreement (which [*], as at the Effective Date are as follows: [*]). 
  

	2.2	 Exclusions to Licensed Data 

The Parties acknowledge that the Licensed Data shall be provided to Licensee in anonymized form, except as provided in this Section 2.2.
Nothing in this Agreement shall oblige UCLB to disclose or provide to Licensee any data which constitutes “personal data” or “sensitive personal data” under the United Kingdom’s Data Protection Act 1998 (as amended) or which
would constitute “personal data” under the General Data Protection Regulation 2016/679 and/or any implementing or replacement legislation applicable in the United Kingdom from time to time during the Term, and any such data is excluded
from the Licensed Data and the scope of the rights granted to Licensee under this Agreement; it being understood and agreed that the Parties shall from time to time negotiate mutually satisfactory agreements that provide for the sharing of the data
on a non-anonymized basis if, but only to the extent, compliant with all of the applicable laws referred to in this Section 2.2. 
  

	2.3	 Reserved Rights  

The license granted in Section 2.1 hereof is expressly made subject to the following: 

(a)    The license granted in Section 2.1 is subject to the following rights retained by each Licensor and by each of
their Affiliates, in UCLB’s case including but not limited to UCL, to: 
  

	 	(i)	 publish the general scientific findings from research conducted in whole or in part by any Licensor or by UCL
as part of the Study or using or otherwise related to the Licensed Data and/ or Patent Rights following [*] prior written notice to the Licensee; 

  

	 	(ii)	 use, practice, or transfer the Licensed Data and Patent Rights for research, teaching and other
educationally-related purposes including performance of the Study; and 

  

	 	(iii)	 permit all other non-profit and/or academic research institutions the
right to use the Licensed Data and Patent Rights, and to practice use of any Licensed Data and Patent Rights, for such organizations’ internal non-commercial research purposes. 

(b)    The license granted in Section 2.1 is subject to a non-exclusive,
irrevocable, royalty-free license heretofore granted to the U.S. Government. The Parties agree that, notwithstanding any use of descriptive terms such as “exclusive” in this Agreement, the U.S. Government has certain rights in the Licensed
Data and/or Patent Rights as set forth in 37 CFR 401. Licensee agrees to comply with all obligations resulting from such government rights, including, but not limited to, the requirement that any products sold in the United States based upon such
technology must be substantially manufactured in the United States to the extent required by 35 U.S.C. Sec. 204. Kansas will offer reasonable assistance in obtaining a waiver of the requirements set forth in this Section 2.4 upon
Licensee’s request where such waiver is applicable. 
  

	2.4	 Affiliates 

Licensee may extend the license granted herein to any Affiliate if the Affiliate consents in writing to be bound by this Agreement to the same
extent as Licensee. Licensee shall ensure that its Affiliates comply fully with the terms of this Agreement and shall be responsible for any breach of or non-compliance with this Agreement by its Affiliates as
if the breach or non-compliance had been a breach of or non-compliance with this Agreement by Licensee. Licensee shall ensure that if any Affiliate ceases to be an
Affiliate as a result of a change of Control or otherwise, 

  
 4 

[*] = Certain confidential information contained in this document, marked by brackets, is omitted because it is both (i) not material and (ii) would be
competitively harmful if publicly disclosed. 

 
that former Affiliate immediately upon such cessation (i) ceases all use or exploitation of the Licensed Data and/or Patent Rights and ceases developing, manufacturing, having manufactured,
using, selling and/or having sold Licensed Products, and (ii) returns to Licensee or destroys any documents or other materials in the former Affiliate’s possession or under its control and that contain the Licensed Data or the
Licensors’ or UCL’s confidential information. 
  

	2.5	 Sublicensing 

Each Licensor hereby grants to Licensee the right to enter into sublicensing agreements with Sublicensees, provided that Licensee shall
(i) notify each Licensor in writing of every sublicense agreement and each amendment thereto within [*] after their execution, and indicate the name of the Sublicensee, the territory of the sublicense, the scope of the sublicense, and [*]
thereunder, and (ii) upon request, provide each Licensor with a copy of each sublicense granted by Licensee hereunder, and any amendments thereto or terminations thereof. Licensee shall ensure that each such sublicense agreement shall include
obligations on the Sublicensee which are consistent with the obligations on Licensee under this Agreement and prohibits the Sublicensee from granting further sublicenses of its rights in relation to the Licensed Data and/or Patent Rights to any
third party (other than distributors and wholesalers as part of any marketing of the Licensed Product, in which case such distributors and wholesalers shall be deemed to be “Sublicensees” under this Agreement). If this Agreement is
terminated for any reason, [*]. Licensee shall, prior to execution of each sublicense, make the intended Sublicensee(s) aware of this contingency. Licensee shall ensure that each Sublicensee complies fully with the terms of the relevant sublicensing
agreement and shall be responsible for any breach of or non-compliance with a sublicensing agreement by its Sublicensees as if the breach or non-compliance had been a
breach of or non-compliance with this Agreement by Licensee. 
  

	2.6	 IND Transfer 

Kansas shall (a) at Licensee’s option, either close or inactivate each of the IND(s) for such Licensed Product, or transfer such
IND(s) to Licensee, and (b) with Licensee’s input, complete all relevant activities related to such IND as required for Licensee to assume regulatory ownership, as applicable, all within [*] after Licensee’s notice, including the
delivery of true, complete, and executed copies of (i) a letter from Kansas to the FDA confirming transfer of the IND to Licensee (which letter shall provide that the transfer shall be effective as of December 14, 2017), (ii) Form 1571,
(iii) Form 1572, (iv) Form 3674, and (v) any relevant IRB compliance documents (collectively, the “IND Transfer”), all of which shall be attached hereto as Exhibit C and the information therein shall be true and correct as of
December 14, 2017, the effective date of transfer. Licensee shall retain legal counsel to provide direction to Kansas and coordinate all steps of the IND Transfer. Licensee shall bear all costs related to the IND Transfer. Subject to compliance
by Kansas with this Agreement, Licensee shall ensure that the IND Transfer is implemented in a timely manner and that it shall not affect the progress of the Study. 

ARTICLE 3. TERM OF AGREEMENT 
  

	3.1	 This Agreement shall be in full force and effect, on a country-by-country basis, for the period from the Effective Date until the date that is ten (10) years from the first commercial sale of the Licensed Product in such country (on a country-by-country basis, the “Term”), unless otherwise terminated by operation of law or by acts of the Parties pursuant to the terms of this Agreement.

  

	3.2	 The license rights granted to Licensee under Section 2.1 shall terminate upon termination (but not
expiration) of this Agreement in accordance with the terms of this Agreement, and Licensee shall thereafter (and ensure that its Affiliates and Sublicensees) make no further use of the Licensed Data and/or Patent Rights whatsoever and to the extent
that the Licensed Data is not in the public domain as at the date of termination, shall (and ensure that its Affiliates and Sublicensees) promptly destroy all copies of the Licensed Data in its (or their) possession (other than the retention of one
copy for recording keeping purposes). Upon expiration of the Term, Licensee shall have a fully-paid up, irrevocable, royalty-free, exclusive license in and to and under the Licensed Data and Patent Rights to research, develop, make, have made, use,
offer for sale, sell, import and/or otherwise commercialize the Licensed Product in the Field of Use throughout the Territory. 

  
 5 

[*] = Certain confidential information contained in this document, marked by brackets, is omitted because it is both (i) not material and (ii) would be
competitively harmful if publicly disclosed. 

 ARTICLE 4. ROYALTIES 

 

	4.1	 Royalty 

As consideration for the licenses under this Agreement, Licensee shall pay to the applicable Licensors in the aggregate an earned royalty of
two percent (2%) of Net Sales, and each of KUCTC and UCLB, as applicable, shall receive [*] of each such royalty payment. Earned royalties shall accrue in each country for the duration of the Term in such country. For clarity, any royalties shall
only be paid once with respect to the sale of any Licensed Product (and, therefore, will not cover any resales). 
 ARTICLE 5.
COMMERCIAL DILIGENCE & MILESTONES 
  

	5.1	 Commercial Diligence 

Upon execution of this Agreement, Licensee shall use Commercially Diligent Efforts to develop, manufacture, practice, sell and use the Licensed
Products in order to make them readily available to the general public. In addition, Licensee shall use Commercially Diligent Efforts to perform the development plan for the Study attached as Exhibit B, subject to performance by each of the
Licensors of the matters contemplated by the development plan. 
  

	5.2	 Reporting 

Licensee shall provide to each Licensor [*] a written report setting out (in reasonable detail) (i) all activities conducted by Licensee
and its Affiliates and Sublicensees since the Effective Date or since the date of the previous report (as appropriate) to develop, manufacture, practice, sell and use the Licensed Products in order to make them readily available to the general
public, and (ii) the current and projected activities being taken or planned to be taken by Licensee and its Affiliates and Sublicensees to bring Licensed Products to market, and to maximize the sale of Licensed Products in the Territory. 

 

	5.3	 Annual Meeting 

Licensee will on either Licensor’s request meet (in person or by tele or video conference) with the relevant Licensor at least once per
calendar year to discuss progress with regard to the development and commercialisation of Licensed Products and Licensee’s efforts to maximize sales of Licensed Products in the Territory. 

 

	5.4	 Delivery of the Study Report 

Licensee shall deliver to each Licensor a copy of the final clinical study report for the Study within [*] following the [*] of the Study. 

 

	5.5	 Inclusion of Licensed Data in NDA and MAA and pricing and reimbursement approvals 

Licensee covenants and agrees that it shall promptly withdraw all references to Licensed Data (other than with respect to references solely for
purposes as safety data) in all New Drug Applications or Marketing Authorisation Applications (or other comparable filings) and/or pricing and reimbursement approvals (or applications therefor) filed by or on behalf of Licensee, any Sublicensee, or
their respective Affiliates following any termination (other than expiry) of this Agreement. 
  

	5.6	 Reporting for Impact Purposes 

Licensee acknowledges that part of each Licensors’ purpose in licensing the Licensed Data and/or Patent Rights to Licensee pursuant this
Agreement is to ensure that the Licensed Data and/or Patent Rights are made available for use and commercial exploitation with the intention of benefitting society and the economy. In order to enable each Licensor to monitor the benefit that they
are providing, and to enable each Licensor to demonstrate the impact of its research activities, to society and the economy, Licensee will provide to each Licensor on [*] during the Term and for a period of [*] thereafter a written report describing
in reasonable detail how it has used the Licensed Data and/or Patent Rights and the societal and economic benefits generated therefrom. Licensee acknowledges that each Licensor shall be entitled to make use of any written reports received from
Licensee (and the information contained therein) pursuant to this Section 5.6 in applications for research or other grant related funding and in submissions to Higher Education funding bodies such as HEFCE and/ or HEIF (or any replacements for
either of those entities) and like entities. 

  
 6 

[*] = Certain confidential information contained in this document, marked by brackets, is omitted because it is both (i) not material and (ii) would be
competitively harmful if publicly disclosed. 

 ARTICLE 6. EQUITY OWNERSHIP 

 

	6.1	 Stock 

(a)    In consideration of the rights granted to Licensee by each of the Licensors in this Agreement (and solely in the
case of KLSDC but not UCLB, following completion of the IND Transfer), Licensee will issue or otherwise deliver to KLSDC and UCLB, as applicable, ordinary shares of Licensee (being class A-shares if the
Licensee’s shares are not listed or authorized for trading as further set out below) with a nominal value of DKK 1.00 per share (the “Shares”) in accordance with this Article 6.1. Such Shares will be issued or registered, as
applicable, in the name of KLSDC and UCLB, as applicable. 
 (b)    Subject to Article 6.1(f), following the end of each
calendar year, Licensee shall issue or otherwise deliver to KLSDC and UCLB, as applicable, a total number of Shares equal to: 
  

	 	(i)	 The aggregate amount of cash funding the Licensors reported to have spent under the Grant in the
Licensors’ reports to the FDA of funds spent under the Grant (hereinafter the “Reports”) submitted during the preceding calendar year converted into DKK at a fixed exchange rate of [*] DKK per 1 USD (which amount, in the aggregate
(i.e. under all Reports, for all calendar years aggregated, and for KLSDC and UCLB combined) shall be capped at the DKK equivalent of USD 2,500,000 (Two Million Five Hundred Thousand Dollars) using the same exchange rate) 

divided by 
  

	 	(ii)	 the DKK value attributed to each Share (in accordance with Article 6.1 (c)) below: 

(c)    The DKK value attributed to each Share shall be: 

 

	 	(i)	 if Licensee’s ordinary shares are not listed or authorized for trading on any recognized national
securities exchange (including NASDAQ, NYSE, or NASDAQ Copenhagen) at the date of the issuance of the Shares (the “Relevant Date”), [*] DKK; or 

  

	 	(ii)	 if Licensee’s ordinary shares are listed or authorized for trading on any recognized national securities
exchange (including NASDAQ, NYSE, or NASDAQ Copenhagen) at the Relevant Date, the average closing price in DKK of Licensee’s ordinary shares on the applicable national securities exchange for the [*] days immediately preceding (and not
including) the Relevant Date. 

 For the avoidance of doubt, Licensee shall issue or otherwise deliver [*] of the total
number of Shares to KLSDC and [*] of the total number of Shares to UCLB. 
 By way of example, if (a) the Licensee’s ordinary
shares are listed or authorized for trading on NASDAQ Copenhagen and the average closing price of Licensee’s ordinary shares for the [*] days immediately preceding the date of issuance was DKK [*], and (b) the amount of the reported Grant
spending was DKK[*], then each of KLSDC and UCLB would receive [*] ordinary shares. 
 (d)    To the extent possible
under applicable law, the Licensee shall issue or otherwise deliver the Shares to KLSDC and UCLB [*]. Hence, to the extent allowed under the Danish Companies Act (in Danish “Selskabsloven”), the Shares shall: (i) be delivered to KLSDC
and UCLB by the Licensee [*] (i.e. sale of Licensee’s own Shares for DKK [*] to KLSDC and UCLB); or (ii) be issued by the Licensee (without pre-emption rights being taken up by the other
shareholders) [*], in accordance with section 153(1)(ii) and section 165 the Danish Companies Act or (iii) a combination of (i) and (ii). 

(e)    If the Shares cannot be issued or otherwise delivered [*] to KLSDC and UCLB, KLSDC and UCLB shall - at their sole
discretion - be entitled to: (i) subscribe for the Shares [*]; or (ii) terminate this Agreement in accordance with Article 12 below. 

  
 7 

[*] = Certain confidential information contained in this document, marked by brackets, is omitted because it is both (i) not material and (ii) would be
competitively harmful if publicly disclosed. 

 (f)    The Shares shall be issued or otherwise delivered to KLSDC and
UCLB, as applicable, no later than [*] provided that the Licensee has received: (i) a written notification from one or both Licensor(s) stating that (a) Licensors have submitted the Report(s) to the FDA detailing the amount of funds spent
under the Grant and (b) such Report(s) were submitted in accordance with the applicable FDA standards, and (ii) a copy of such Report(s) submitted to the FDA, certified by an officer of the University of Kansas Medical Center Research
Institute, Inc. (KUMCRI) to be true and complete as of such date. 
 (g)    Notwithstanding anything contained herein to
the contrary: (i) Licensee shall only be obligated to issue or otherwise deliver Shares to Licensors one (1) time during any twelve (12) month period and (ii) in the event any of the Share calculations contemplated above result
in a fractional Share amount, such fractional Share shall be rounded to the nearest whole number. 
 (h)    This Article
6 shall replace, supersede and cancel Articles 3.3 - 3.7 of the option agreement dated [*] entered into between the Parties regarding a Phase II clinical trial for testing arimoclomol in sIBM, and any delivery of Shares under this License Agreement
shall fulfil in its entirety Licensee’s obligations to this effect under such option agreement. 
 ARTICLE 7. CONFIDENTIALITY

  

	7.1	 Licensee and each Licensor acknowledge that either Party may provide certain information to the other with
regard to the Licensed Product that is considered to be confidential. Licensee and each Licensor shall take all reasonable precautions to protect such confidential information. Such precautions shall involve at least the same degree of care and
precaution that the recipient customarily uses to protect its own confidential information, but in no circumstance less than reasonable care. 

  

	7.2	 Licensee acknowledges that each Licensor is subject to the Kansas Open Records Act, K.S.A. 45-215 et seq. Each Licensor shall keep confidential any information provided to each Licensor by Licensee that Licensee considers confidential, to the extent allowable under the Kansas Open Records Act.

  

	7.3	 The provisions of Section 7.1 shall not apply to confidential information which the relevant Party can
demonstrate by reasonable written evidence: 

  

	 	a.	 was, prior to its receipt under this Agreement, in the possession of that Party and at its free disposal; or

  

	 	b.	 is subsequently disclosed to the relevant Party by a third party without any obligations of confidence; or

  

	 	c.	 is or becomes generally available to the public through no act or default of the relevant Party or its agents,
employees, Affiliates or Sublicensees; 

  

	 	d.	 the relevant Party is required to disclose by or to the courts of any competent jurisdiction, or to any
government regulatory agency or financial authority, provided that the Receiving Party shall (i) inform the disclosing Party as soon as is reasonably practicable of such requirement, and (ii) at the disclosing Party’s request and cost
seek to persuade the court, agency or authority to have the information treated in a confidential manner, where this is possible under the court, agency or authority’s procedures; or 

 

	 	e.	 which a Party is advised by its information officer that it is required to disclose under the Freedom of
Information Act 2000 or the Environmental Information Regulations 2004. 

 ARTICLE 8. QUARTERLY & ANNUAL
REPORTS 
  

	8.1	 Annual and Quarterly Royalty Report 

Within [*] days after the calendar year in which Net Sales first occur, and within [*] days after each calendar quarter thereafter, Licensee
shall provide each Licensor with a written report detailing all sales and uses, if any, made of Licensed Products during the preceding calendar quarter, and detailing the amount of Net Sales made during such quarter by Licensee and each of its
Affiliates and Sublicensees, broken down on a country-by country basis, and calculating the royalties due to each Licensor pursuant to Article 4 hereof. Each report shall include at least the following: 

 

	 	a.	 number or volume of Licensed Products manufactured, leased and sold by and/or for Licensee, Affiliates and all
Sublicensees; 

  
 8 

[*] = Certain confidential information contained in this document, marked by brackets, is omitted because it is both (i) not material and (ii) would be
competitively harmful if publicly disclosed. 

	 	b.	 accounting for Net Sales, noting the deductions applicable as provided in Section 1.13;

  

	 	c.	 total royalties due to KUCTC and UCLB, as applicable; and 

 

	 	d.	 names and addresses of all Affiliates and Sublicensees. 

Each report shall be in substantially similar form as Exhibit “A” attached hereto. Each such report shall be signed by an officer of
Licensee (or the officer’s designee). With each such report submitted, Licensee shall pay to each Licensor the royalties and fees due and payable under this Agreement. If no royalties shall be due, Licensee shall so report. Licensee’s
failure to submit a royalty report in the required form will constitute a breach of this Agreement. Licensee will continue to deliver royalty reports to each Licensor after the termination or expiration of this Agreement until such time as all
Licensed Product(s) permitted to be sold after termination have been sold or destroyed and all corresponding royalties have been paid to Licensors. 
  

	8.2	 Licensee shall provide Licensor with Licensee’s audited financial statements for the preceding fiscal year
including, at a minimum, a balance sheet and income statement (i) in the case Licensee’s ordinary shares are not listed or authorized for trading on any recognized national securities exchange (including NASDAQ, NYSE, or NASDAQ
Copenhagen), on or before the [*] day following the close of Licensee’s fiscal year or (ii) in the case Licensee’s ordinary shares are listed or authorized for trading on any recognized national securities exchange (including NASDAQ,
NYSE, or NASDAQ Copenhagen), within [*] days following the date Licensee’s audited financial statements are required to be publicly filed or otherwise provided to shareholders. 

 

	8.3	 In addition to the regular reports required by Section 8.1 and 8.2 hereof, Licensee shall provide a
written report to each Licensor of the date of first occurrence of Net Sales in each country within [*] days of the occurrence thereof. 

ARTICLE 9. PAYMENTS, RECORDS and AUDITS 
  

	9.1	 Payments 

Licensee shall pay all royalties accruing to KUCTC and UCLB, as applicable, [*] within [*] days following the calendar quarter in which Net
Sales occur. Each payment to KUCTC will reference the KUCTC Technology [*]. All cash payments to KUCTC and UCLB, as applicable, will be made [*]: 

in the case of KUCTC, payable by wire transfer or check to the [*] and sent to: 

[*] 
 Wire Transfer Information:

 [*] 
 in the case of UCLB,
payable by wire transfer to : 
 [*] 

For converting any Net Sales made in a currency other than [*], the Parties will use the conversion rate customarily used by the Licensee,
Affiliate or Sublicensee at such time for preparation of its relevant financial statements. 
  

	9.2	 Deductions 

All sums due under this Agreement will be made without any set-off, deduction or withholding except as
may be required by law. If Licensee is required by law to make any deduction or to withhold any part of any amount due to either of the Licensors under this Agreement, Licensee will give to the relevant Licensor proper evidence of the amount
deducted or withheld and payment of that amount to the relevant taxation authority, and will do all things in its power to enable or assist the relevant Licensor to claim exemption from or, if that is not possible, to obtain a credit for the amount
deducted or withheld under any applicable double taxation or similar agreement from time to time in force. 

  
 9 

[*] = Certain confidential information contained in this document, marked by brackets, is omitted because it is both (i) not material and (ii) would be
competitively harmful if publicly disclosed. 

	9.3	 Late Payments 

In the event royalty payments or other fees are not received by [*] or [*], as applicable, when due hereunder, Licensee shall pay to the
applicable Licensors interest charges at the rate of [*] per annum on the total royalties or fees due for the reporting period. 
  

	9.4	 Records 

Licensee shall keep, and cause its Sublicensees and Affiliates to keep, complete, true and accurate records and books (in accordance with
international accounting or U.S. GAAP standards) containing all particulars that may be necessary for the purpose of showing the amounts payable to the applicable Licensors hereunder. Records and books shall be kept at Licensee’s or the
Affiliate’s or Sublicensee’s principal place of business. 
  

	9.5	 Audit 

Such books and the supporting data shall be open to inspection by each Licensor or its agents, upon reasonable prior notice to Licensee, at all
reasonable times during the Term, upon reasonable prior notice to Licensee, for the purpose of verifying Licensee’s royalty statement or compliance in other respects with this Agreement. Such access will be available to each Licensor upon not
less than [*] days written notice to Licensee, not more than once each calendar year of the Term, during normal business hours, and once a year for [*] years after the expiration or termination of this Agreement. Licensee shall co-operate with each Licensor in good faith to resolve any discrepancies identified during any such inspection and shall pay any shortfall in the amounts paid to each Licensor under this Agreement, together with
interest on late payment as specified in Section 9.3, within [*] days following receipt of a copy of the inspection report. Should such inspection lead to the discovery of a greater than [*] discrepancy in reporting to any Licensor’s
detriment, Licensee agrees to pay the full cost of such inspection. 
 ARTICLE 10. PATENT MARKING 

 

	10.1	 Licensee shall permanently and legibly mark all Licensed Products made, used or sold under the terms of this
Agreement, or their containers, in accordance with all applicable patent-marking and notice provisions under Title 35, United States Code. 

ARTICLE 11. PATENT PROSECUTION AND MAINTENANCE 
  

	11.1	 Licensee shall be responsible for the drafting, filing, prosecution and maintenance of any Patent Rights (in
the name of each Licensor, as appropriate) at its own cost and expense. Licensee shall consult with each Licensor in making any material decisions relating to the Patent Rights such as in which countries to file and maintain the Patent Rights, and
the scope of the claims, and any reductions or limitations to the extent of the patent coverage. 

  

	11.2	 Licensee will ensure that each Licensor receives copies of all material correspondence to and from patent
offices in respect of the Patent Rights, including copies of all documents generated in or with such correspondence, and where practicable shall be given reasonable notice of and the opportunity to participate in any material conference calls or
meetings with Licensee’s patent attorneys in relation to the drafting, filing, prosecution and maintenance of the Patent Rights, so that Licensors may be informed of progress with the drafting, filing, prosecution and maintenance of the
Patents. Licensee shall promptly notify each Licensor of the grant of each patent comprised within the Patents. 

  

	11.3	 If Licensee wishes to abandon any application or not to maintain any patent contained with the Patent Rights,
it shall give no less than [*] prior written notice to each Licensor and on the expiry of such notice period the licenses of the relevant Patent Rights granted to the Licensee under this Agreement shall cease. 

ARTICLE 12. TERMINATION BY LICENSOR 
  

	12.1	 If Licensee should: (a) fail to make any payment at the time that the same should be due; (b) violate
or fail to perform any covenant, condition, or undertaking of this Agreement to be performed by it hereunder in any material respect, including any breach of Article 6 (including, for the avoidance of doubt, section 6.1(e)); (c) file a bankruptcy
action, or have a bankruptcy action against it, or become Insolvent; or (d) enter into a composition with creditors, or have a receiver appointed for it; then any Licensor may give written notice of such default to Licensee. If Licensor gives

  
 10 

[*] = Certain confidential information contained in this document, marked by brackets, is omitted because it is both (i) not material and (ii) would be
competitively harmful if publicly disclosed. 

	 	
notice of a default under (a) or (b) and Licensee should fail to cure such default within [*] days following the date of such notice, each Licensor shall have the right to terminate this
Agreement forthwith by written notice to the other Parties. If Licensor gives notice of a default under (c) or (d) this Agreement shall forthwith terminate. 

  

	12.2	 No termination of this Agreement by any Licensor shall relieve Licensee of its obligation to pay any monetary
obligation or to issue the shares to the Licensors pursuant to Article 6 due or owing at the time of such termination, and shall not impair any accrued right of any Licensor. Licensee shall [*] and [*] or [*]. Articles 4 (in relation to any payments
due to Licensors in relation to the period prior to termination and in relation to any disposition of Licensed Products following termination pursuant to Article 14), 6, 7, 8 (in relation to any reports due to Licensors in relation to the period
prior to termination or in relation to any disposition of Licensed Products following termination pursuant to Article 14), 9, 17, 20, 21, 22, 23, 24, 26 and 27 and Section 2.5 (in relation only to Licensee’s obligation to assign its right,
title and interest to any sublicenses to Licensors on termination), 3.2, 5.4, 5.6, 12.2, 13.3, 15.2, 15.3, 15.4, and 15.5 hereof shall survive any termination of this Agreement. 

 

	12.3	 In case this Agreement is terminated, any Shares that should have been delivered/issued to the Licensors by the
Licensee in accordance with Article 6 had the Agreement not been terminated (i.e. based on Reports submitted by either of the Licensors after 1 January of the year of termination and up until and including the date of termination), shall be
issued or delivered by the Licensee to the Licensors no later than [*] calendar days after the date of termination of this Agreement. To the extent the Licensee cannot issue or deliver such Shares to the Licensors in accordance with Article 6.1(d)
under applicable law, the Licensors shall be entitled (but not obligated) to subscribe for such Shares at par value. 

ARTICLE 13. TERMINATION BY LICENSEE 
  

	13.1	 Subject to section 13.4, if any of the Licensors should: (a) violate or fail to perform any covenant,
condition, or undertaking of this Agreement to be performed by it hereunder in any material respect; (b) file a bankruptcy action, or have a bankruptcy action against it, or become Insolvent; or (c) enter into a composition with creditors,
or have a receiver appointed for it; then Licensee may give written notice of such default to each Licensor. If Licensee gives notice of a default under (a) and the relevant Licensor should fail to cure such default within [*] days following
the date of such notice, Licensee shall have the right to terminate this Agreement forthwith by written notice to the other Parties. If Licensee gives notice of a default under (c) or (d) this Agreement shall forthwith terminate.

  

	13.2	 Following the delivery to the Licensors of the final clinical study report for the Study, but subject always to
section 13.4, Licensee may terminate this Agreement at any time and from time to time thereafter without cause, by giving written notice thereof to each Licensor if, but only if: (a) the Study fails to achieve the primary end points as per the
protocol accepted by the KUMC internal review board (IRB), (b) any applicable regulatory authority notifies Licensee, Sublicensee, or any of their Affiliates that it has declined to approve the New Drug Application or Marketing Authorisation
Application (or other comparable filings) for the Licensed Product for the Intended Indication, or (c) such regulatory authority has approved or indicates it will approve such New Drug Application or Marketing Authorisation Application (or
other comparable filings) with a product label for the Licensed Product for the Intended Indication which must contain a so-called “Black-Box Warning.” Such
termination shall be effective [*] days after such notice and all Licensee’s rights associated therewith shall cease as of that date. 

  

	13.3	 Any termination pursuant to Section 13.1 or 13.2 hereof shall not relieve Licensee of its obligation to
pay any monetary obligation or to issue the shares to the Licensors pursuant to Article 6 due or owing at the time of such termination, and shall not impair any accrued right of any Licensor. Licensee shall [*] and [*] or [*]. Articles 4 (in
relation to any payments due to Licensors in relation to the period prior to termination and in relation to any disposition of Licensed Products following termination pursuant to Article 14), 6, 7, 8 (in relation to any reports due to Licensors in
relation to the period prior to termination or in relation to any disposition of Licensed Products following termination pursuant to Article 14), 9, 17, 20, 21, 22, 23, 24, 26 and 27and Section 2.5 (in relation only to Licensee’s
obligation to assign its right, title and interest to any sublicenses to Licensors on termination), 3.2, 5.4, 5.6, 12.2, 13.3, 15.2, 15.3, 15.4, and 15.5 hereof shall survive any termination of this Agreement. 

 

	13.4	 Notwithstanding any other provision of this Agreement, Licensee shall not be entitled to exercise any right to
terminate this Agreement until such times as the Study has been completed and all shares to be issued to Licensors pursuant to Article 6 based on [*] have been duly issued to the Licensors [*]. 

  
 11 

[*] = Certain confidential information contained in this document, marked by brackets, is omitted because it is both (i) not material and (ii) would be
competitively harmful if publicly disclosed. 

 ARTICLE 14. DISPOSITION OF LICENSED PRODUCTS ON HAND 

 

	14.1	 Upon expiration or termination of this Agreement by either Party, Licensee shall be entitled to dispose of any
such Licensed Products following such expiration or termination subject to payment of royalties to the Licensors under Article 4. 

ARTICLE 15. WARRANTY BY LICENSOR 
  

	15.1	 Each Licensor warrants that it has the lawful right to grant the licenses set forth in this Agreement. UCLB
hereby warrants that UCL has assigned to UCLB all of UCL’s right, title and interest in and to any data and know-how generated in the course of research in connection with the Study undertaken at UCL.

  

	15.2	 EXCEPT AS EXPRESSLY PROVIDED IN SECTION 15.1, THE PARTIES ACKNOWLEDGE AND AGREE THAT EACH LICENSOR, ITS
AFFILIATES, AGENTS, EMPLOYEES, AND THE INVENTORS HAVE MADE NO REPRESENTATIONS OR WARRANTIES, EXPRESS OR IMPLIED, INCLUDING BUT NOT LIMITED TO, WARRANTIES OF MERCHANTABILITY OR FITNESS FOR A PARTICULAR PURPOSE, OR ABSENCE OF LATENT OR OTHER DEFECTS,
WHETHER OR NOT DISCOVERABLE. ANY CONDITIONS, WARRANTIES OR OTHER TERMS IMPLIED BY STATUTE OR COMMON LAW ARE HEREBY EXCLUDED FROM THIS AGREEMENT TO THE FULLEST EXTENT PERMITTED BY LAW. IN NO EVENT SHALL ANY LICENSOR, ITS AFFILIATES, AGENTS,
EMPLOYEES, AND THE INVENTORS BE HELD RESPONSIBLE FOR ANY DIRECT, SPECIAL, INDIRECT OR CONSEQUENTIAL DAMAGES OR LOSS OF PROFIT (WHETHER DIRECT OR INDIRECT) OR LOSS OF REVENUE, BUSINESS OPPORTUNITY OR GOODWILL ARISING OUT OF OR IN CONNECTION WITH THE
EXERCISE OF THE RIGHTS GRANTED TO LICENSEE UNDER THIS AGREEMENT, EVEN IF ANY LICENSOR IS ADVISED IN ADVANCE OF THE POSSIBILITY OF SUCH DAMAGES. 

  

	15.3	 Nothing in this Agreement shall be construed as: 

 

	 	a.	 a warranty or representation by any Licensor or their Affiliates as to the conduct of the Study or the efficacy
or suitability of the Licensed Data and/or Patent Rights for any particular purpose, notwithstanding that the Licensee’s intended purpose may be known to the Licensor and/ or their Affiliates. 

 

	 	b.	 a warranty or representation by any Licensor and/ or their Affiliates that the exercise or practice by the
Licensee of the license of the Licensed Data and/or Patent Rights granted herein (including for the purpose of making, using, selling, offering for sale, or importing the Licensed Product) is or will be free from infringement of intellectual
property rights of third parties. 

  

	 	c.	 as to the scope of any of the Patent Rights or that any of the Patent Rights is or will be valid or (in the
case of an application) will proceed to grant. 

  

	 	d.	 an obligation by any Licensor or their Affiliates to bring or prosecute actions or suits against third parties
for patent infringement. 

  

	 	e.	 conferring by implication, estoppel or otherwise any license or rights under any patents or other intellectual
property rights of any Licensor other than the Licensed Data and/or Patent Rights. 

  

	15.4	 Each Licensor’s total cumulative liability in connection with this Agreement, whether in contract or tort
or otherwise, will not exceed [*] or [*], whichever is the lower. Notwithstanding the foregoing, the liability of KUMC, as an agency of the State of Kansas, is defined under and limited by the Kansas Tort Claims Act (K.S.A. 75-6101 et seq.). 

  

	15.5	 Nothing in this Agreement excludes any Party’s liability to the extent that it may not be so excluded
under applicable law, including but not limited to any such liability for death or personal injury caused by that Party’s negligence, or liability for fraud or fraudulent misrepresentation. 

  
 12 

[*] = Certain confidential information contained in this document, marked by brackets, is omitted because it is both (i) not material and (ii) would be
competitively harmful if publicly disclosed. 

 ARTICLE 16. PATENT ENFORCEMENT 

 

	16.1	 Enforcement 

As between the Parties, Licensee shall have the sole right, but not the obligation, to institute, prosecute, and control at its own expense any
action or proceeding with respect to any matters relating to Patent Rights by counsel of its own choice, in Licensee’s own name and under Licensee’s direction and control. The foregoing right of Licensee shall include the right to perform
all actions of a reference product sponsor set forth in the U.S. Hatch-Waxman Act or Public Health Service Act, and any ex-U.S. equivalent of such laws. 

 

	16.2	 Right to Participate; Joinder 

Each Licensor agrees, at the reasonable request [*], to join any such action or proceeding as a party if doing so is necessary for the purposes
of establishing standing or is otherwise required by applicable Law to pursue such action or proceeding contemplated by Section 16.1, provided always that (i) before commencing any such action, Licensee shall consult with Licensors as to
the advisability of the action, its effect on the good name of the Licensors, the public interest, and how the action should be conducted, (ii) each Licensor shall be indemnified and secured by Licensee in a reasonable manner as to any costs
(including internal costs), damages, expenses or other liability which may be incurred as a result of so joining any such action) and shall have the right to be separately represented by its own counsel [*], and (iii) Licensee shall pay to the
Licensors royalties, in accordance with Article 4, on any damages received from such action as if the amount of such damages after deduction of all Parties’ reasonable expenses in relation to the action were Net Sales. Notwithstanding the
foregoing, neither Licensor shall be obliged to join any suit or to take any action in its own name if that Licensor has reasonable grounds to believe that the action is inadvisable or is likely to damage their good name provided that where a
Licensor notifies Licensee that it declines to join any suit or take any action in its own name on the foregoing grounds and Licensee considers that it cannot effectively enforce the Patent Rights or obtain effective relief in the relevant
jurisdiction without the joinder of the relevant Licensor, then the relevant Licensor and Licensee will work together in good faith to try to identify a way for the Licensee to enforce such rights or obtain such relief in another manner. 

 

	16.3	 Settlement 

A settlement or consent judgment or other voluntary final disposition of a suit under this Section 16.3 may be entered into without the
consent of the Party not bringing suit; provided, however, that any such settlement, consent judgment or other disposition of any action or proceeding by a Party under this Section 16.3 shall not, without the consent of the Party not bringing
suit, impose any liability on any of the Licensors or contain any allegation or admission of wrongdoing relating to a Party or otherwise contain any statements relating to that Party which may damage that Party’s reputation without that
Party’s prior written consent. 
 ARTICLE 17. INSURANCE 

 

	17.1	 Insurance Requirements 

Licensee will, at its sole cost and expense and at all times during the Term, procure and maintain commercial general liability insurance
issued by an insurance carrier with [*] or better in amounts not less than [*] per incident and [*] annual aggregate. Licensee will have [*]. All rights of subrogation will be waived against Licensor and its insurers. Such commercial general
liability and product liability insurance will provide (i) product liability coverage; (ii) broad form contractual liability coverage for Licensee’s indemnification under this Agreement; and (iii) coverage for litigation costs.
The specified minimum insurance amounts will not constitute a limitation on Licensee’s obligation to indemnify each of the Licensors, their Affiliates and their respective officers, employees and agents, under this Agreement. 

 

	17.2	 Evidence of Insurance and Notice of Changes 

Licensee will provide each Licensor with written evidence of such insurance upon request by the relevant Licensor. Licensee will provide each
Licensor with written notice of at least [*] days prior to the cancellation, non-renewal, or material change in such insurance. 

  
 13 

[*] = Certain confidential information contained in this document, marked by brackets, is omitted because it is both (i) not material and (ii) would be
competitively harmful if publicly disclosed. 

	17.3	 Continuing Insurance Obligations 

Licensee will maintain such commercial general liability insurance beyond the expiration or termination of this Agreement during (i) the
period that any Licensed Data is being used or Licensed Product(s) is being commercially distributed or sold by Licensee, any Affiliate, or any Sublicensee or agent of Licensee; and (ii) for [*] years after such period. 

ARTICLE 18. WAIVER 
  

	18.1	 No waiver by any Party hereto of any breach or default of any of the covenants or agreements herein set forth
shall be deemed a waiver as to any subsequent and/or similar breach or default. 

 ARTICLE 19. ASSIGNABILITY

  

	19.1	 This Agreement (and any rights and/ or obligations under this Agreement) are not assignable or otherwise
transferable (including by operation of law, merger, or other business combination) by Licensee without the prior written consent of each Licensor; it being understood and agreed that any Party may assign its rights and obligations under this
Agreement to any Affiliate so long as the original Party remains obligated to the other Parties as if no such assignment has occurred. The failure of Licensee to comply with the terms of this paragraph shall be grounds for termination of the
Agreement by any Licensor under Article 12. 

 ARTICLE 20. INDEMNIFICATION BY LICENSEE 

 

	20.1	 Licensee shall indemnify, hold harmless and defend each Licensor, and its respective officers, employees,
inventors, Affiliates, and agents, against any and all claims, suits, losses, damages, costs, liabilities, fees and expenses (including reasonable fees of attorneys) resulting from or arising out of or in connection with: (a) the exercise of
any license granted under this Agreement; (b) the breach of this Agreement by Licensee or its Affiliates or Sublicensees; (c) Licensee’s failure to comply with any applicable laws, rules, or regulations, or (d) any act, error, or
omission of Licensee, its officers, agents, employees, Affiliates, or Sublicensees, except where such claims, suits, losses, damages, costs, fees, or expenses result solely from the gross negligence, fraud, or intentional misconduct of the relevant
Licensor, or its Affiliates, officers, employees or agents. 

  

	20.2	 Licensee shall give each Licensor prompt and timely notice of any claim or suit instituted of which Licensee
has knowledge that in any way, directly or indirectly, affects or might affect each Licensor, and each Licensor shall have the right at its own expense to participate in the defense of the same. 

ARTICLE 21. NOTICES 
  

	21.1	 Any payment, notice or other communication required or permitted to be given to any Party hereto shall be in
writing and shall be deemed to have been properly given and effective: (a) on the date of delivery if delivered in person during recipient’s normal business hours; or (b) on the date of attempted delivery if delivered by international
courier, express mail service or first-class mail, registered or certified. Such notice shall be sent or delivered to the respective addresses given below, or to such other address as the relevant Party shall designate by written notice given to the
other Parties as follows: 

 In the case of Licensee: 

Orphazyme A/S 
 Ole Maaløes
Vej 3, DK-2200 N 
 Copenhagen, Denmark 

With a copy to: 
 Dechert LLP 

1900 K St., N.W. 
 Washington,
D.C. 20006 
 Telephone: (202) 261-3440 

Attn: David E. Schulman 

  
 14 

[*] = Certain confidential information contained in this document, marked by brackets, is omitted because it is both (i) not material and (ii) would be
competitively harmful if publicly disclosed. 

 In the case of Licensors: 

[*] 
 and: 

[*] 
 ARTICLE 22. REGULATORY
COMPLIANCE 
  

	22.1	 When required by local/national law, Licensee shall register this Agreement, [*], and otherwise ensure that the
local/national laws affecting this Agreement are fully satisfied. 

  

	22.2	 Licensee shall comply, in all material respects, with all applicable U.S. laws dealing with the export and/or
management of technology or information. Licensee understands that the Arms Export Control Act (AECA), including its implementing International Traffic In Arms Regulations (ITAR,) and the Export Administration Act (EAA), including its Export
Administration Regulations (EAR), are some (but not all) of the laws and regulations that comprise the U.S. export laws and regulations. Licensee further understands that the U.S. export laws and regulations include (but are not limited to): (1)
ITAR and EAR product/service/data-specific requirements; (2) ITAR and EAR ultimate destination-specific requirements; (3) ITAR and EAR end user-specific requirements; (4) ITAR and EAR end
use-specific requirements; (5) Foreign Corrupt Practices Act; and (6) anti-boycott laws and regulations. Licensee will comply with all then-current applicable export laws and regulations of the U.S.
Government (and other applicable U.S. laws and regulations) pertaining to the Licensed Product(s) and/or Patent Rights (including any associated products, items, articles, computer software, media, services, technical data, and other information).
Licensee certifies that it will not, directly or indirectly, export (including any deemed export), nor re-export (including any deemed re-export) the Licensed Product(s)
and/or Patent Rights (including any associated products, items, articles, computer software, media, services, technical data, and other information) in violation of U.S. export laws and regulations or other applicable U.S. laws and regulations.
Licensee will include an appropriate provision in its agreements with its authorized Sublicensees to assure that these parties comply with all then-current applicable U.S. export laws and regulations and other applicable U.S. laws and regulations

  

	22.3	 If required by applicable law, then Licensee agrees that products used or sold in the United States embodying
Licensed Products shall be manufactured substantially in the United States, unless a written waiver is obtained in advance from the sponsoring federal agency. 

 

	22.4	 General Compliance with Laws 

Licensee will at all times (and will ensure its Affiliates and Sublicensees) comply with all legislation, rules, regulations and statutory
requirements applying to and obtain any consents necessary for its use of the Licensed Data and/or Patent Rights and/or the development, manufacture, use and sale of Licensed Products in any country within the Territory. 

 

	22.5	 Bribery Act 

Licensee shall (and shall procure that any persons associated with it engaged in the performance of this Agreement including its Affiliates and
Sublicensees shall): 
  

	 	a.	 comply with all applicable laws and codes of practice relating to anti-bribery and anti-corruption including
but not limited to the United Kingdom Bribery Act 2010 and without prejudice to the foregoing generality, shall not engage in any activity, practice or conduct which would constitute an offence under sections 1, 2 or 6 of such Act do or omit to do
any act that will cause or lead UCLB to be in breach of that Act; 

  

	 	b.	 comply with UCLB’s ethics, anti-bribery and anti-corruption policies as available on UCL’s website
from time to time and maintain in place and enforce throughout the term of this Agreement adequate procedures to ensure compliance with sub-section a. above; and 

 

	 	c.	 promptly report to UCLB any request or demand for any undue financial or other advantage of any kind received
in connection with the performance of this Agreement. 

  
 15 

[*] = Certain confidential information contained in this document, marked by brackets, is omitted because it is both (i) not material and (ii) would be
competitively harmful if publicly disclosed. 

 For the purpose of this Section, the meaning of adequate procedures and whether a person is
associated with another person shall be determined in accordance with the United Kingdom Bribery Act 2010 (and any guidance issued under section 9 of that Act). Breach of this Section shall be deemed a material breach of this Agreement entitling
UCLB to terminate under Article 11. 
  

	22.6	 Modern Slavery Act 

Licensee shall (and shall procure that any persons associated with it engaged in the performance of this Agreement including its Affiliates and
Sublicensees shall) comply with all applicable laws and codes of practice relating to anti-slavery including the united Kingdom Modern Slavery Act 2015. Such compliance shall include ensuring that all reasonable steps are taken to ensure that all
parties associated with the development, manufacture and commercialisation of the Licensed Products comply with all applicable laws and codes of practice relating to anti-slavery including the United Kingdom Modern Slavery Act 2015. 

 

	22.7	 Export Control Regulations 

Licensee shall ensure that, in using the Licensed Data and/or Patent Rights and in selling Licensed Products, it and its Affiliates, employees,
sub-contractors and Sublicensees shall comply fully with any United Nations trade sanctions or EU or UK legislation or regulation, from time to time in force (as applicable), which impose arms embargoes or
control the export of goods, technology or software, including weapons of mass destruction and arms, military, paramilitary and security equipment and dual-use items (items designed for civil use but which can
be used for military purposes) and certain drugs and chemicals. 
 ARTICLE 23. GOVERNING LAW 

23.1    This Agreement shall be interpreted and construed in accordance with the laws of [*], without application of any principles of
choice of laws. 
 ARTICLE 24. RELATIONSHIP OF PARTIES 

 

	24.1	 In assuming and performing the respective obligations under this Agreement, Licensee and each Licensor are each
acting as independent parties and neither shall be considered or represent itself as a joint venture, partner, agent or employee of the other. 

  

	24.2	 Licensee acknowledges that the obligations on Kansas and UCLB under this Agreement shall be several. Nothing in
this Agreement is intended to create joint liability or joint and several liability between the Licensors, and neither Kansas nor UCL shall have any responsibility for the acts and omissions of the other under or in connection with this Agreement,
or for the other’s breach or non-performance of the warranties, undertaking and obligations contained in this Agreement. 

ARTICLE 25. USE OF NAMES 
  

	25.1	 By Licensee 

Licensee shall not, without prior written consent of any Licensor, use the name or any trademark or trade name owned by such Licensor in any
publication, publicity, advertising, or otherwise, except that Licensee may identify such Licensor as the licensor of the Licensed Data, Patent Rights and Licensed Products. 
  

	25.2	 By Licensor 

Each Licensor may use Licensee’s name in connection with such Licensor’s publicity related to such Licensor’s intellectual
property and commercialization achievements. 
 ARTICLE 26. DISPUTE RESOLUTION 

 

	26.1	 Except for the right of any Party to apply to a court of competent jurisdiction for a temporary restraining
order, a preliminary injunction, or other equitable relief to preserve the status quo or prevent irreparable harm, any and all claims, disputes or controversies arising under, out of, or in connection with the Agreement which the Parties shall

  
 16 

[*] = Certain confidential information contained in this document, marked by brackets, is omitted because it is both (i) not material and (ii) would be
competitively harmful if publicly disclosed. 

	 	
be unable to resolve within [*] days shall be mediated in good faith. The Party raising such dispute shall promptly advise each other Party of such dispute. By not later than [*] business days
after the recipient has received such notice of dispute, each Party shall have selected for itself a representative who shall have the authority to bind such Party, and shall additionally have advised each other Party in writing of the name and
title of such representative. By not later than [*] days after the date of such notice of dispute, the Party against whom the dispute shall be raised shall select a mediator [*] and such representatives shall schedule a date with such mediator for a
hearing. The Parties shall enter into good faith mediation and shall share the costs equally. If the representatives of the Parties have not been able to resolve the dispute within [*] business days after such mediation hearing, then any and all
claims, disputes or controversies arising under, out of, or in connection with this Agreement, shall be resolved through arbitration if [*], or through any judicial proceeding either in the courts of [*] or [*], to whose jurisdiction for such
purposes Licensee and each Licensor each hereby irrevocably consents and submits. All costs and expenses, including reasonable attorneys’ fees, of each Party in connection with resolution of a dispute by arbitration or litigation of such
controversy or claim shall be borne as directed by the court, judicial body or arbitrator presiding over the relevant proceedings. Subject to sections 12.2 and 13.3, if the court, judicial body or arbitrator does not make such a direction, each
Party will be responsible for its own costs and expenses, including attorneys’ fees. 

 ARTICLE 27. GENERAL
PROVISIONS 
  

	27.1	 The headings of the several sections are inserted for convenience of reference only and are not intended to be
a part of or to affect the meaning or interpretation of this Agreement. 

  

	27.2	 This Agreement shall not be binding upon the Parties until it has been signed below by or on behalf of each
Party. 

  

	27.3	 No amendment or modification of this Agreement shall be valid or binding upon the Parties unless made in
writing and signed by both Parties hereto. 

  

	27.4	 This Agreement embodies the entire understanding of the Parties and supersedes all previous communications,
representations or understandings, either oral or written, between the Parties relating to the subject matter thereof. For the avoidance of doubt this Agreement shall not supersede the terms of the Material Transfer Agreement between UCL, KUMC and
Licensee dated [*] (as amended) which shall remain in full force and effect. 

  

	27.5	 The provisions of this Agreement are severable, and in the event that any provision of this Agreement shall be
determined to be invalid or unenforceable under any controlling body of the law, such invalidity or unenforceability shall not in any way affect the validity or enforceability of the remaining provisions hereof. 

 

	27.6	 This Agreement may be signed in counterparts, each of which when taken together shall constitute one fully
executed document. 

  

	27.7	 In the event of any litigation, arbitration, judicial reference or other legal proceeding involving the Parties
to this Agreement to enforce any provision of this Agreement, to enforce any remedy available upon default under this Agreement, or seeking a declaration of the rights of either Party under this Agreement, the prevailing Party shall be entitled to
recover from the other [*] as may be determined by the court, judicial body or arbitrator presiding over the relevant proceedings. 

  

	27.8	 Except as required by applicable law, legal process, or stock exchange rules, none of the Parties may disclose
the financial terms of this Agreement without the prior written consent of the other Party. 

  

	27.9	 Licensee acknowledges that each of KUCTC and KLSDC is a 501c(3) corporation; Licensee will exercise necessary
precautions to ensure that each of KUCTC and KLSDC’s 501c(3) status is not jeopardized by Licensee’s activity under this Agreement by refraining from engaging in prohibited activity, including, but not limited to, making political
contributions or engaging in substantial lobbying of legislators for or on behalf of KUCTC and KLSDC. 

  
 17 

[*] = Certain confidential information contained in this document, marked by brackets, is omitted because it is both (i) not material and (ii) would be
competitively harmful if publicly disclosed. 

 IN WITNESS WHEREOF, each Licensor and Licensee have executed this Agreement by their
respective officers hereunto duly authorized, on the day and year hereinafter written. 
  

									
	ORPHAZYME A/S	  		  	KU CENTER FOR TECHNOLOGY COMMERCIALIZATION, INC.
					
	By	 	 /s/ Georges Gemayel
	  		  	By:	 	
                     
                                         
                   

		 	(Signature)	  		  		 	(Signature)
					
	Name	 	 GEORGES Gemayel
	  		  	Name:	 	[*]
		 	(Please Print)	  		  		 	
					
	Title	 	 Chairman
	  		  	Title:	 	[*]
					
	Date	 	 Oct 25, 2017
	  		  	Date	 	  

			
	UNIVERSITY OF KANSAS	  		  	 KANSAS LIFE SCIENCES DEVELOPMENT

COMPANY, INC.

					
	By	 	  
	  		  	By:	 	  

		 	(Signature)	  		  		 	(Signature)
					
	Name	 	  
	  		  	Name:	 	  

		 	(Please Print)	  		  		 	
		 		  		  	Title:	 	  

	Title	 	  
	  		  		 	
		 		  		  	Date	 	  

	Date	 	  
	  		  		 	
				
	UCL BUSINESS PLC	  		  		 	
					
	By	 	  
	  		  		 	
		 	(Signature)	  		  		 	
					
	Name	 	  
	  		  		 	
		 	(Please Print)	  		  		 	
					
	Title	 	  
	  		  		 	
					
	Date	 	  
	  		  		 	

 [Signature page to license agreement] 

  
 [*] = Certain confidential information
contained in this document, marked by brackets, is omitted because it is both (i) not material and (ii) would be competitively harmful if publicly disclosed. 

 IN WITNESS WHEREOF, each Licensor and Licensee have executed this Agreement by their
respective officers hereunto duly authorized, on the day and year hereinafter written. 
  

									
	ORPHAZYME A/S	  		  	KU CENTER FOR TECHNOLOGY COMMERCIALIZATION, INC.
					
	By	 	 /s/ Anders Hinsby
	  		  	By:	 	 [*]

		 	(Signature)	  		  		 	(Signature)
					
	Name	 	 ANDERS HINSBY
	  		  	Name:	 	[*]
		 	(Please Print)	  		  		 	
					
	Title	 	 CEO
	  		  	Title:	 	[*]
					
	Date	 	 26 Oct 2017
	  		  	Date	 	 [*]

			
	UNIVERSITY OF KANSAS	  		  	 KANSAS LIFE SCIENCES DEVELOPMENT

COMPANY, INC.

					
	By	 	 [*]
	  		  	By:	 	 [*]

		 	(Signature)	  		  		 	(Signature)
					
	Name	 	 [*]
	  		  	Name:	 	 [*]

		 	(Please Print)	  		  		 	
					
	Title	 	 [*]
	  		  	Title:	 	 [*]

					
	Date	 	 [*]
	  		  	Date	 	 [*]

				
	UCL BUSINESS PLC	  		  		 	
					
	By	 	 [*]
	  		  		 	
		 	(Signature)	  		  		 	
					
	Name	 	 [*]
	  		  		 	
		 	(Please Print)	  		  		 	
					
	Title	 	 [*]
	  		  		 	
					
	Date	 	 [*]
	  		  		 	

 [Signature page to license agreement] 

  
 [*] = Certain confidential information
contained in this document, marked by brackets, is omitted because it is both (i) not material and (ii) would be competitively harmful if publicly disclosed. 

 EXHIBIT “A” 

XXXX ROYALTY REPORT 
 LICENSEE:
                                         
                                         
                                   KUCTC Technology ID #
                                 

Period Covered: From
                             Through:
                             

Prepared By:
                                         
                                         
                                 Date:
                             

Approved By:
                                         
                                         
                               Date:
                             

If Licensee has several licensed products, please prepare separate reports for each. Then, compile all licensed products into a summary report. 

 

																																					
	 Country and
Patent
	  	Product or
Tradename	 	  	Quantity
Sold	 	  	Unit
Price	 	  	Gross
Sales	 	  	* Less
Allowances	 	  	Net
Sales	 	  	Royalty
Rate	 	  	Period Royalty Amount	 
	  	This Year	 	  	Last Year	 
	 USA
	  	 	            	 	  	 	            	 	  	 	            	 	  	$	             	 	  	$	             	 	  	$	             	 	  	 	            	 	  	$	             	 	  	$	             	 
	 #
	  				  				  				  				  				  				  				  				  			
	 Canada
	  				  				  				  				  				  				  				  				  			
	 #
	  				  				  				  				  				  				  				  				  			
	 Europe:
	  				  				  				  				  				  				  				  				  			
	 #
	  				  				  				  				  				  				  				  				  			
	 #
	  				  				  				  				  				  				  				  				  			
	 #
	  				  				  				  				  				  				  				  				  			
	 #
	  				  				  				  				  				  				  				  				  			
	 #
	  				  				  				  				  				  				  				  				  			
	 #
	  				  				  				  				  				  				  				  				  			
	 Japan
	  				  				  				  				  				  				  				  				  			
	 #
	  				  				  				  				  				  				  				  				  			
	 Other:
	  				  				  				  				  				  				  				  				  			
	 #
	  				  				  				  				  				  				  				  				  			
	 #
	  				  				  				  				  				  				  				  				  			
	 Sublicense #1 (name)
	  				  				  				  				  				  				  				  				  			
	 #
	  				  				  				  				  				  				  				  				  			
	 Sublicense #2 (name)
	  				  				  				  				  				  				  				  				  			
	 #
	  				  				  				  				  				  				  				  				  			
		  	  
	  
	 	  	  
	  
	 	  	  
	  
	 	  	  
	  
	 	  	  
	  
	 	  	  
	  
	 	  	  
	  
	 	  	  
	  
	 	  	  
	  
	 
	 TOTAL:
	  				  				  				  	$	 	 	  	$	 	 	  	$	 	 	  				  	$	 	 	  	$	 	 
		  	  
	  
	 	  	  
	  
	 	  	  
	  
	 	  	  
	  
	 	  	  
	  
	 	  	  
	  
	 	  	  
	  
	 	  	  
	  
	 	  	  
	  
	 

 Total Royalty Due:
$                                         
            
 The following royalty forecast is
non-binding and for internal planning only: 
 Royalty Forecast Under This Agreement: Qtr
1:                     Qtr
2:                     Qtr
3:                     Qtr
4:                     
  

	*	 On a separate page, please indicate the reasons for adjustments, if significant. Please refer to the following
examples as applicable: (1) cash, trade or quantity discounts actually allowed; (2) sales, use, tariff, customs duties or other excise taxes directly imposed upon particular sales; (3) outbound transportation charges—prepaid or
allowed, and (4) allowances or credits to third parties for rejections or returns. 

  
 [*] = Certain confidential information
contained in this document, marked by brackets, is omitted because it is both (i) not material and (ii) would be competitively harmful if publicly disclosed. 

 EXHIBIT “B” 

DEVELOPMENT PLAN 
 [*] 

  
 [*] = Certain confidential information
contained in this document, marked by brackets, is omitted because it is both (i) not material and (ii) would be competitively harmful if publicly disclosed. 

 EXHIBIT “C” 

IND TRANSFER LETTERS 
 [*] 

  
 [*] = Certain confidential information
contained in this document, marked by brackets, is omitted because it is both (i) not material and (ii) would be competitively harmful if publicly disclosed.

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