U.S. dollars by personal check, bank draft, or money order payable to the Company, by money transfer or direct account debits;

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, and the Company shall so certify upon Purchaser’s request.

U.S. Revolving Loans. Prior to the Termination Date, each U.S. Lender, by its acceptance hereof, severally and not jointly agrees, subject to the terms and conditions hereof, to make a revolving loan or loans (each individually a “U.S. Revolving Loan” and, collectively, the “U.S. Revolving Loans”) in U.S. Dollars to the Borrowers from time to time on a revolving basis up to the amount of such Lender’s U.S. Revolving Credit Commitment in effect at such time; provided, however, the sum of the aggregate principal amount of U.S. Revolving Loans, U.S. Swing Loans and U.S. L/C Obligations at any time outstanding shall not exceed the sum of all U.S. Revolving Credit Commitments in effect at such time. Each Borrowing of U.S. Revolving Loans shall be made ratably by the U.S. Lenders in proportion to their respective U.S. Revolver Percentages. As provided in Section 2.4(a), and subject to the terms hereof, the Borrowers may elect that each Borrowing of U.S. Revolving Loans be either Base Rate Loans or EurodollarTranche Rate Loans. U.S. Revolving Loans may be repaid and reborrowed before the Termination Date, subject to the terms and conditions hereof.

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. 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. Real Property Holding Corporation. Neither the Company nor any of its subsidiaries is, or has ever been, and so long as the Note is held by the Buyer, shall become, a U.S. real property holding corporation within

U.S. Tax Consequences. Upon vesting of Shares, Participant will include in taxable income the difference between the fair market value of the vesting Shares, as determined on the date of their vesting, and the price paid for the Shares. This will be treated as ordinary income by Participant and will be subject to withholding by the Company when required by applicable law. In the absence of an Election (defined below), the Company shall withhold a number of vesting Shares with a fair market value (determined on the date of their vesting) equal to the minimum amount the Company is required to withhold for income and employment taxes. If Participant makes an Election, then Participant must, prior to making the Election, pay in cash (or check) to the Company an amount equal to the amount the Company is required to withhold for income and employment taxes.

U.C.C. Search Results, etc. The [[Administrative Agent:Organization]] shall have received: