Exhibit 10.1

FIRST AMENDMENT TO LICENSE AGREEMENT

This First Amendment (“Amendment”) is made and entered into as of March 1, 2014
(the “Amendment Effective Date”) by and between St. Jude Children’s Research
Hospital, having its principal place of business at 262 Danny Thomas Place,
Memphis, Tennessee 38105 (“INSTITUTION”), and Ultragenyx Pharmaceutical Inc., a
Delaware corporation having a principal place of business at 60 Leveroni Court,
Novato, CA 94949 (“LICENSEE”).

Agreement

Whereas, LICENSEE and INSTITUTION entered into the certain License Agreement
effective September 1, 2012 (the “License Agreement”);

Whereas, the parties desire to amend the License Agreement as specified herein.

Now, Therefore, in consideration of the mutual covenants and for other good and
valuable consideration, the receipt and sufficiency of which are hereby
acknowledged, LICENSEE and INSTITUTION hereby agree as follows:

1.All capitalized terms used in this Amendment and not otherwise defined herein
shall have the same meaning as defined in the License Agreement.

2.The following new Section 3.4 is hereby added after the end of Section 3.3 of
the License Agreement:

“3.4As between the Parties, INSTITUTION shall have the first right to prosecute
and maintain the Licensed Patents worldwide at its own cost and
expense.  INSTITUTION will notify LICENSEE of any decision not to continue to
prosecute, maintain or pay the expenses of prosecution or maintenance of any
Licensed Patent.  Notwithstanding the foregoing, with respect to the prosecution
and maintenance of that certain Licensed Patent with the International
Application Number PCT/US2012/052629 (the “Licensee Directed Prosecution”), (a)
LICENSEE will reimburse INSTITUTION all reasonable out-of-pocket fees associated
with the Licensee Directed Prosecution in the European Union, Japan, Australia
and Canada (collectively, the “629 Ex-US Patent Expenses”); and (b) INSTITUTION
will (i) keep LICENSEE advised of the status of all communications and actual
and prospective filings regarding the Licensee Directed Prosecution, (ii) give
LICENSEE a reasonable opportunity (but in no event less than thirty (30)
business days) to review and comment on any such communications and filings
proposed to be sent to any applicable patent authority with respect to the
Licensee Directed Prosecution and (iii) reasonably consider in good faith all
comments of LICENSEE before making any such communication or filing.  As long as
LICENSEE is reimbursing the 629 Ex-US Patent Expenses in accordance with this
Section 3.4, INSTITUTION shall continue and shall not cease the Licensee
Directed Prosecution in the European Union, Japan, Australia

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and Canada.  Any such 629 Ex-US Patent Expenses paid by LICENSEE pursuant to
this Section 3.4 shall be fully creditable against any future payment that may
be due by LICENSEE under any license agreement entered into between the parties
in accordance with Section 3.3.”

3.Each party hereby represents and warrants to the other that it has the power
and authority to enter into this Amendment and this Amendment constitutes a
legal, valid and binding obligation, enforceable against such party in
accordance with its terms.

4.This Amendment shall be effective upon the Amendment Effective Date set forth
above.

5.Except as expressly modified herein, all of the terms and conditions of the
License Agreement shall remain in full force and effect.  To the extent that
there are any inconsistencies between this Amendment and the License Agreement,
the terms of this Amendment shall govern and shall supersede the License
Agreement.

6.This Amendment shall be interpreted and enforced in accordance with the laws
of the State of New York, without reference to principles of conflicts of laws.

7.This Amendment may be executed in two (2) or more counterparts, each of which
shall be deemed an original, but both of which together shall constitute one and
the same instrument.  Each Party may execute this Amendment by facsimile
transmission or in AdobeÔ Portable Document Format (PDF) sent by electronic
mail.  Facsimile or PDF signatures of authorized signatories of the Parties will
be deemed to be original signatures, will be valid and binding upon the Parties,
and, upon delivery, will constitute due execution of this Amendment.

[Signature Page Follows]

 

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In Witness Whereof, the parties have caused this Amendment to be executed by
their duly authorized representatives as of the Amendment Effective Date set
forth above.

 

 

 

Ultragenyx Pharmaceutical Inc.St Jude Children’s Research Hospital

 

By:   /s/ Tom KassbergBy:   /s/ J. Scott Elmer

 

Name:  Tom KassbergName:  J. Scott Elmer

 

Title:  CBOTitle:  Director, Technology Licensing