Exhibit 10.1

Certain information in this document has been omitted and filed separately with
the Securities and Exchange Commission. Confidential treatment has been
requested with respect to the omitted portions marked [***].

AMENDMENT NO. 5 TO COLLABORATION AND LICENSE AGREEMENT

 

This Amendment No. 5 to the Collaboration and License Agreement (“Amendment”) is
made and entered into by and between Kyowa Hakko Kirin Co., Ltd., a company
organized and existing under the laws of Japan, with an address at 1-9-2
Ohtemachi, Chiyoda-ku, Tokyo, 100-0004, Japan (“KHK”) and Ultragenyx
Pharmaceutical Inc., a company organized and existing under the laws of the
State of Delaware, with an address at 60 Leveroni Court, Novato, California
94949, USA (“UGNX”).

RECITALS

 

 

A.

WHEREAS, KHK and UGNX entered into a Collaboration and License Agreement
effective as of August 29, 2013, an Amendment No. 1 to Collaboration and License
Agreement effective as of August 24, 2015, an Amendment No. 2 to Collaboration
and License Agreement effective as of November 28, 2016, an Amendment No. 3 to
Collaboration and License Agreement effective as of September 29, 2017, and an
Amendment No. 4 to Collaboration and License Agreement effective as of January
29, 2018 (collectively, the “Collaboration Agreement”).

 

 

B.

WHEREAS, both Parties wish to further amend the Collaboration Agreement as set
forth below.

 

 

C.

NOW, THEREFORE, in consideration of the mutual covenants and premises herein
contained, the Parties agree as follows:

 

 

1.

This Amendment shall be effective as of April 30, 2018 (the “Amendment Effective
Date”).

 

 

2.

Any capitalized terms that are not defined in this Amendment will have their
respective meanings set forth in the Collaboration Agreement.

 

 

3.

The definition of “GAAP” in Section 1.1.32 of the Collaboration Agreement is
hereby deleted in its entirety and replaced with the following:

 

1.1.32   “GAAP” means generally accepted accounting principles applicable to a
Party for reporting purposes for their respective countries (e.g. Japanese
Accounting Standards, International Financial Reporting Standards, U.S.
Generally Accepted Accounting Principles) as consistently applied throughout the
applicable periods indicated herein by or on behalf of the relevant Party.

 

 

4.

In the definition of “Net Sales” in Section 1.1.54 of the Collaboration
Agreement, the parenthetical words in the second line of the second paragraph
“(as applicable

 

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Certain information in this document has been omitted and filed separately with
the Securities and Exchange Commission. Confidential treatment has been
requested with respect to the omitted portions marked [***].

 

in the country of sale)” is hereby deleted in its entirety and replaced with the
following:  

 

(as applicable in each country in the Territory or European Territory where the
Parties are required to report the Net Sales in accordance with Section 7.2.4)

 

 

5.

Notwithstanding anything in the Collaboration Agreement to the contrary,
including without limitation, Section 7.2.4 thereof, the reporting and payment
schedules related to Sections 4.9.4, 6.1.7(a), 6.1.7(b), 7.1.1, 7.1.2, 7.2.1,
7.2.2, and 7.2.3 shall be the reporting and payment schedules set forth in
Exhibit A attached hereto.

 

 

6.

For Licensed Products to be supplied to patients in the U.S. as part of a “free”
drug program (i.e. supply program to patients prior to the Licensed Product
being subject to pricing reimbursement for that patient), UGNX shall pay to KHK
the prices set forth in the Side Letter between KHK and UGNX dated August 29,
2013. Additionally, for KRN23 [***] the price shall be [***]/vial.  Such pricing
shall remain fixed for a minimum of [***].  KHK may equitably adjust such
pricing [***], commencing with [***], due to changes in KHK’s manufacturing
costs, by providing UGNX with written notice (including an explanation of and
supporting evidence for (if available) the applicable manufacturing cost
changes) on or before [***] immediately preceding the calendar year subject to
the price adjustment.

 

 

7.

Except as expressly provided in this Amendment, all other terms, conditions and
provisions of the Collaboration Agreement shall continue in full force and
effect as provided therein.

 

 

8.

This Amendment may be executed in identical duplicate copies exchanged by
facsimile or e-mail (PDF form) transmission.  The Parties agree to execute two
identical original copies of this Amendment after exchanging signed facsimile
versions.  Each identical counterpart will be deemed an original, but all of
which together will constitute one and the same instrument.

IN WITNESS WHEREOF, the Parties have executed this Amendment No. 5 to
Collaboration and License Agreement to be effective as of the Amendment
Effective Date.

 

 

 

 

 

 

 

 

 

 

 

KYOWA HAKKO KIRIN CO., LTD.

 

 

 

ULTRAGENYX PHARMACEUTICAL INC.

 

 

 

 

 

By:

 

/s/ Yasuo Fujii

 

 

 

By:

 

/s/ Thomas Kassberg

Name:

 

Yasuo Fujii

 

 

 

Name:

 

Thomas Kassberg

Title:

 

Director, Business Development Dept

 

 

 

Title:

 

Chief Business Officer

2

 

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Certain information in this document has been omitted and filed separately with
the Securities and Exchange Commission. Confidential treatment has been
requested with respect to the omitted portions marked [***].

3

 

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Certain information in this document has been omitted and filed separately with
the Securities and Exchange Commission. Confidential treatment has been
requested with respect to the omitted portions marked [***].

Exhibit A

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