U.K. Lender Status Confirmation. Each U.K. Lender which becomes a U.K. Lender on the date of this Agreement confirms that, at the date of this Agreement, it is a U.K. Qualifying Lender. Each U.K. Lender which becomes a U.K. Lender after the date of this Agreement shall indicate, in the Assignment and Assumption which it executes on becoming a party, which of the following categories it falls within:

U.K. Tax Indemnity. The U.K. Borrower shall (within the later of # 10 days of demand therefor and # three (3[[Holdings:Organization]] Business Days before the relevant loss, liability or cost will be suffered[[Holdings:Organization]] pay to a U.K. Lender or the Administrative Agent an amount equal to the loss, liability or cost that such U.K. Lender or the Administrative Agent (as applicable[[Holdings:Organization]] determines in its reasonable discretion has been (directly or indirectly[[Holdings:Organization]] suffered by such U.K. Lender or the Administrative Agent, as applicable, for or on account of any Tax in respect of the U.K. Facility. Notwithstanding the foregoing, this Section 3.02(I[[Holdings:Organization]](e[[Holdings:Organization]] shall not apply:

U.S. Real Property Holding Corporation. The Company is not and has never been a U.S. real property holding corporation within the meaning of Section 897 of the Internal Revenue Code of 1986, as amended.

“U.S. Launch Plan” means the initial plan and budget for Development and Commercialization activities leading up to commercial launch of Acer Product in the U.S. for UCD and MSUD, which have been exchanged between and agreed to by the Parties prior to the Effective Date.

[U.S. QFC Stay Rules. The parties agree that # to the extent that prior to the date hereof both parties have adhered to the 2018 ISDA U.S. Resolution Stay Protocol (the “Protocol”), the terms of the Protocol are incorporated into and form a part of the Agreement, and for such purposes the Agreement shall be deemed a Protocol Covered Agreement and each party shall be deemed to have the same status as Regulated Entity and/or Adhering Party as applicable to it under the Protocol; # to the extent that prior to the date hereof the parties have executed a separate agreement the effect of which is to amend the qualified financial contracts between them to conform with the requirements of the QFC Stay Rules (the “Bilateral Agreement”), the terms of the Bilateral Agreement are incorporated into and form a part of the Agreement and each party shall be deemed to have the status of “Covered Entity” or “Counterparty Entity” (or other similar term) as applicable to it under the Bilateral Agreement; or # if clause (i) and clause (ii) do not apply, the terms of Section 1 and Section 2 and the related defined terms (together, the “Bilateral Terms”) of the form of bilateral template entitled “Full-Length Omnibus (for use between U.S. G-SIBs and Corporate Groups)” published by ISDA on November 2, 2018 (currently available on the 2018 ISDA U.S. Resolution Stay Protocol page at www.isda.org and, a copy of which is available upon request), the effect of which is to amend the qualified financial contracts between the parties thereto to conform with the requirements of the QFC Stay Rules, are hereby incorporated into and form a part of the Agreement, and for such purposes the Agreement shall be deemed a “Covered Agreement,” Dealer shall be deemed a “Covered Entity” and Dealer shall be deemed a “Counterparty Entity.” In the event that, after the date of the Agreement, both parties hereto become adhering parties to the Protocol, the terms of the Protocol will replace the terms of this Section 8(x) of the Confirmation. In the event of any inconsistencies between the Agreement and the terms of the Protocol, the Bilateral Agreement or the Bilateral Terms (each, the “QFC Stay Terms”), as applicable, the QFC Stay Terms will govern. Terms used in this paragraph without definition shall have the meanings assigned to them under the QFC Stay Rules. For purposes of this paragraph, references to “the Agreement” include any related credit enhancements entered into between the parties or provided by one to the other. In addition, the parties agree that the terms of this paragraph shall be incorporated into any related covered affiliate credit enhancements, with all references to Dealer Parent replaced by references to the covered affiliate support provider.“QFC Stay Rules” means the regulations codified at 12 C.F.R. 252.2, 252.81–8, 12 C.F.R. 382.1-7 and 12 C.F.R. 47.1-8, which, subject to limited exceptions, require an express recognition of the stay-and-transfer powers of the FDIC under the Federal Deposit Insurance Act and the Orderly Liquidation Authority under Title II of the Dodd Frank Wall Street Reform and Consumer Protection Act and the override of default rights related directly or indirectly to the entry of an affiliate into certain insolvency proceedings and any restrictions on the transfer of any covered affiliate credit enhancements.]

U.S. Withholding Tax Exemptions. Any Lender that is entitled to an exemption from or reduction of withholding Tax with respect to payments made under any Loan Document shall deliver to Borrower and the Administrative Agent, at the time or times reasonably requested by Borrower or the Administrative Agent, such properly completed and executed documentation reasonably requested by Borrower or the Administrative Agent as will permit such payments to be made without withholding or at a reduced rate of withholding. In addition, any Lender, if reasonably requested by Borrower or the Administrative Agent, shall deliver such other documentation prescribed by applicable law or reasonably requested by Borrower or the Administrative Agent as will enable Borrower or the Administrative Agent to determine whether or not such Lender is subject to backup withholding or information reporting requirements. Notwithstanding anything to the contrary in the preceding two sentences, the completion, execution and submission of such documentation (other than such documentation set forth in paragraphs [(i), (ii), and (iv) of this Section 3.1(c)]) shall not be required if in the Lender’s reasonable judgment such completion, execution or submission would subject such Lender to any material unreimbursed cost or expense or would materially prejudice the legal or commercial position of such Lender. Without limiting the generality of the foregoing:

“U.S.” means the United States of America, including its territories and possessions (including Puerto Rico).

U.S. Government Rights. The License is expressly subject to all applicable provisions of any license to the United States Government executed by either Penn, CHOP or Institutions and is subject to any overriding obligations to the United States Federal Government under 35 U.S.C. §§200-212, applicable governmental implementing regulations, and the U.S. Government sponsored research agreement or other guidelines, including that products that result from intellectual property funded by the United States Federal Government that are sold in the United States be substantially manufactured in the United States. In the event that Licensee believes in good faith that substantial manufacture of such product is not commercially feasible in the United States and makes a request to Penn in writing to assist in obtaining a waiver of such requirement from the United States Government, then Institutions shall, at the expense of Licensee, use reasonable efforts to assist in obtaining such waiver. In addition, Licensee acknowledges the obligations to the United States Federal Government under 37 Code of Federal Regulations, Part [[Identifier]] and shall provide information to the extent necessary to satisfy any such applicable obligations to the United States Federal Government thereunder.

U.S. Tax Reporting. For the avoidance of doubt, the Borrowers agree not to treat the Term Loan as a “contingent payment debt instrument” for U.S. income tax purposes.

U.S. Government Securities, in each case maturing within one year from the date of acquisition thereof;