Item 1.01 Entry Into a Material Definitive Agreement

Amendment to Loan and Security Agreement

On November 16, 2021 (the "Effective Date"), Scholar Rock Holding Corporation (the "Company" or "we") and Scholar Rock, Inc., a wholly-owned subsidiary of the Company (collectively, the "Borrower"), entered into Amendment No. 1 (the "Amendment No. 1") to the Loan and Security Agreement (the "Loan Agreement") dated October 16, 2020 by and with Oxford Finance LLC ("Collateral Agent") and Silicon Valley Bank ("Bank") (each, a "Lender" and collectively, the "Lenders").

Pursuant to the Loan Agreement, the Company previously received $25.0 million in loan proceeds under Tranche 1 of the Loan Agreement on October 16, 2020. The Company has an additional $25.0 million in loan proceeds available to it if it meets the criteria under Tranche 2 of the Loan Agreement. Amendment No. 1 amended the Loan Agreement to revise the Tranche 2 milestones to be when the Borrower has: (i) publicly announced the Phase 3 clinical trial (SAPPHIRE) design for SRK-015 (Apitegromab) and registered such clinical trial with clinicaltrials.gov; and (ii) initiated Part B of the DRAGON Phase 1 clinical trial for SRK-181.

The foregoing description of Amendment No. 1 does not purport to be complete and is qualified in its entirety by reference to the Amendment No. 1, which we intend to file as an exhibit to our Form 10-K for the year ending December 31, 2021.

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