Scholar Rock Holding Corp

• As of June 30, 2025, Scholar Rock had cash, cash equivalents, and marketable securities of approximately $295 million, which along with cash available to the Company and planned revenues, is expected to fund the anticipated operating and capital expenditure requirements into 2027.

SEC original ↗Scholar Rock Holding Corp — Earnings release (8-K exhibit)EX-99Aug 6, 2025

The Company has primarily financed its operations through various equity financings, the exercise of stock options and warrants, as well as research and development collaboration agreements and the Company s debt facility (Note 11).

SEC original ↗Scholar Rock Holding Corp — 10-Q Quarterly report10-QAug 6, 2026

The following weighted average assumptions were used in determining the fair value of options granted in the six months ended June 30, 2026 and 2025: Six Months Ended June 30, 2026 2025 Risk-free interest rate 3.95 % 4.10 % Expected dividend yield 0.0 % 0.0 % Expected term (in years) 6.04 6.06 Expected volatility 107.10 % 108.33 % 16 Table of Contents 9.

SEC original ↗Scholar Rock Holding Corp — 10-Q Quarterly report10-QAug 6, 2026

Debt On October 16, 2020, the Company entered into a Loan and Security Agreement with Oxford Finance LLC ( Oxford ) and Silicon Valley Bank ( SVB ) for $ 50.0 million (the Loan and Security Agreement ), which was amended in 2022 to increase the Company s borrowing capacity.

SEC original ↗Scholar Rock Holding Corp — 10-Q Quarterly report10-QAug 6, 2026

17 Table of Contents On September 29, 2025, the Company received $ 50.0 million from the next available tranche under the Oxford Loan Agreement, bringing the total borrowings to $ 100.0 million.

SEC original ↗Scholar Rock Holding Corp — 10-Q Quarterly report10-QAug 6, 2026

The 2026 Loan Agreement provides for up to $ 350.0 million (each, a Term Loan and together, the Term Loans ), of which $ 100.0 million of the initial term loan (the Initial Term Loan ) was received in February 2026.

SEC original ↗Scholar Rock Holding Corp — 10-Q Quarterly report10-QAug 6, 2026

htm EX-99.1 Exhibit 99.1 Exhibit 99.1 Scholar Rock Reports Second Quarter 2025 Financial Results and Highlights Business Progress • FDA accepted the apitegromab BLA under priority review with a PDUFA target action of September 22, 2025; finalizing U.

SEC original ↗Scholar Rock Holding Corp — Earnings release (8-K exhibit)EX-99Aug 6, 2025

--(BUSINESS WIRE)— August 6, 2025-- Scholar Rock (NASDAQ: SRRK), a late-stage biopharmaceutical company focused on developing and commercializing apitegromab for patients with spinal muscular atrophy (SMA) and other severe and debilitating neuromuscular diseases, today reported financial results and updates for the second quarter ended June 30, 2025.

SEC original ↗Scholar Rock Holding Corp — Earnings release (8-K exhibit)EX-99Aug 6, 2025

“Our BLA is progressing under priority review towards our September 22 PDUFA date, and our team is working with urgency to prepare to serve children and adults living with spinal muscular atrophy,” said David L.

SEC original ↗Scholar Rock Holding Corp — Earnings release (8-K exhibit)EX-99Aug 6, 2025

, we are continuing to advance our MAA in the EU in parallel, while also planning for extensive global expansion to serve the SMA community with apitegromab worldwide.

SEC original ↗Scholar Rock Holding Corp — Earnings release (8-K exhibit)EX-99Aug 6, 2025

The FDA has accepted the Biologics License Application (BLA) for apitegromab under priority review and has assigned a Prescription Drug User Fee Act (PDUFA) Exhibit 99.1 target action date for apitegromab of September 22, 2025.

SEC original ↗Scholar Rock Holding Corp — Earnings release (8-K exhibit)EX-99Aug 6, 2025

All such forward-looking statements are based on management's current expectations of future events and are subject to a number of risks and uncertainties that could cause actual results to differ materially and adversely from those set forth in or implied by such forward-looking statements.

SEC original ↗Scholar Rock Holding Corp — Earnings release (8-K exhibit)EX-99Aug 6, 2025

Some of the risks and uncertainties that may cause our actual results, performance or achievements to differ materially from those expressed or implied by forward-looking statements include, among others, the following: the timing, scope, or likelihood of our ability to obtain and maintain regulatory approval from the U.

SEC original ↗Scholar Rock Holding Corp — 10-Q Quarterly report10-QAug 6, 2026

Other sections of this report may include additional factors that could adversely affect our business and financial performance.

SEC original ↗Scholar Rock Holding Corp — 10-Q Quarterly report10-QAug 6, 2026

Given these risks and uncertainties, investors should not place undue reliance on forward-looking statements as a prediction of actual results.

SEC original ↗Scholar Rock Holding Corp — 10-Q Quarterly report10-QAug 6, 2026

Investors should also refer to our most recent Annual Report on Form 10-K and our Quarterly Reports on Form 10-Q for future periods and Current Reports on Form 8-K as we file them with the United States Securities and Exchange Commission (the SEC ), and to other materials we may furnish to the public from time to time through Current Reports on Form 8-K or otherwise, for a discussion of risks and uncertainties that may cause actual results, performance or achievements to differ materially from those expressed or implied by forward-looking statements.

SEC original ↗Scholar Rock Holding Corp — 10-Q Quarterly report10-QAug 6, 2026

Information that is based on estimates, forecasts, projections, market research or similar methodologies is inherently subject to uncertainties, and actual events, circumstances or numbers, including actual disease prevalence rates and market size, may differ materially from the information reflected in this Quarterly Report.

SEC original ↗Scholar Rock Holding Corp — 10-Q Quarterly report10-QAug 6, 2026