A.

WHEREAS, KHK and UGNX entered into a Collaboration and License Agreement effective as of August 29, 2013 (the “Original Agreement”) and an Amendment No.1 to Collaboration and License Agreement effective as of August 24, 2015 (together with the Original Agreement, the “Agreement”).

B.

WHEREAS, both Parties wish to further amend the 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 November 28, 2016 (the “Amendment Effective Date”).

2.

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

3.

The following words shall be added at the end of Section 1.1.72 of the Original Agreement:

4.

Notwithstanding anything contained in the Agreement (including Section 3.2 of the Original Agreement) to the contrary, as of the Amendment Effective Date, the Parties hereby agree to disband the JDC and JCC and establish a subcommittee named Joint Core Team subcommittee (“JCT”) instead. The JCT will consist of the same number of representatives designated by each Party, which number will initially be at least seven (7) each for seven (7) functions (PPM, Clinical Science, Commercial, Medical Affairs, Regulatory, CMC, Clinical Operations), provided

that at least one (1) of each representative shall be responsible for one (1) function. The number of each Party’s representatives in the JCT thereafter may be changed by mutual written agreement between the Parties. Without changing the total number of its representatives in the JCT, each Party will be free to change its representatives in the JCT by giving written notice to the other Party. Accordingly, all of the obligations and responsibilities of the JCC and JDC shall be assumed by the JCT which shall be a “Committee” under the Agreement. For clarity, except as expressly provided in this Amendment, the word “JDC” (together with “Joint Development Committee”) and “JCC” (together with “Joint Commercialization Committee”) in the Original Agreement (including Sections 3.2, 3.3, 3.4, 3.5 and 3.6) are hereby all eliminated, and the word “JCT” (together with “Joint Core Team”) are substituted, as of the Amendment Effective Date.

5.

Notwithstanding anything contained in the Agreement to the contrary, the Parties hereby agree that if UGNX requests to conduct Clinical Trials in any country or region outside the Territory and the European Territory for supporting the Core Development Activities, UGNX shall submit to the JSC a written request to add such country or region as an additional territory (each a “New Territory”) and such request shall be promptly reviewed by the JSC. A New Territory shall be finally added as a further amendment to the Appendix attached hereto by the Parties’ mutual written agreement. To the extent necessary for supporting Core Development Activities, KHK will grant to UGNX a non-exclusive, royalty-free license under the Licensed Technology to conduct the Clinical Trials in the Field in such New Territory. For clarity, as of the Amendment Effective Date, the Parties hereby agree that Australia shall be added as a New Territory and therefore, KHK grants UGNX a non-exclusive, royalty-free license under the Licensed Technology to conduct the Clinical Trials in the Field in Australia.

6.

With respect to the Clinical Trials conducted in the New Territory, the Parties hereby acknowledge and agree as follows;

7.

Notwithstanding anything in the Agreement to the contrary, the Parties hereby agree that, to the extent permitted under Applicable Laws, each of them shall promptly take all necessary actions (if required) to have Kyowa Kirin International Plc. (“KKI”) which is a wholly owned subsidiary of KHK or KKI’s Affiliate designated by KKI be (a) the party submitting the Marketing Authorization Application (“MAA”) in the European Territory and (b) the Marketing Authorization Holder (“MAH”) for any Marketing Approval in the European Territory; provided, however, UGNX shall continue to have responsibility for the activities specified as below;

8.

Except as expressly provided in this Amendment, all other terms, conditions and provisions of the Agreement shall continue in full force and effect as provided therein. Capitalized terms used in this Amendment that are not otherwise defined herein shall have the same meanings as such terms are defined in the Agreement.

9.

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.

KYOWA HAKKO KIRIN CO., LTD.

ULTRAGENYX PHARMACEUTICAL INC.

By:

/s/ Tamao Watanabe

By:

/s/ Tom Kassberg

Name:

Tamao Watanabe

Name:

Tom Kassberg

Title:

Director,

Business Development Department

Title:

CBO

country or region as a New Territory

date

1

Australia

November 28, 2016