Scholar Rock Holding Corporation · NASDAQ:SRRK

Scholar Rock — Investor Presentation (July 2026)

33 slides from Scholar Rock's investor presentation, July 2026

1. Slide 1: Scholar Rock Corporate Presentation

   Scholar Rock Corporate Presentation July 2026

2. Slide 2: Forward-Looking Statements

   Forward-Looking Statements Various statements in this presentation concerning the future expectations, plans and prospects of Scholar Rock Holding Corporation and Scholar Rock, Inc. (collectively, “Scholar Rock”), including without limitation, Scholar Rock’s expectations regarding its growth, strate

3. Slide 3: Scholar Rock is poised for a transformative year

   Scholar Rock is poised for a transformative year S H A P I N G T H E F U T U R E O F T R E AT M E N T FOR PATIENTS LIVING WITH RARE NEUROMUSCULAR DISEASES A P I T EG RO M A B FO R PAT I E N T S W I T H S M A U N D E R W AY ONGOING 1 PHASE 2 OPAL SC APITEGROMAB Myostatin inhibitor with a successful P

4. Slide 4: Apitegromab poised to usher in next phase of innovation in SMA

   Apitegromab poised to usher in next phase of innovation in SMA Targeting muscle—the principal organ affected in SMA INITIAL PHASE OF INNOVATION: MOTOR NEURON NEXT PHASE OF INNOVATION: MUSCLE 2016 2019 2020 2025 2026 2030+ Apitegromab U.S. launch Apitegromab European launch Apitegromab global expansi

5. Slide 5: Global apitegromab opportunity in SMA alone offers potential for many years of sustainable growth

   Global apitegromab opportunity in SMA alone offers potential for many years of sustainable growth 1 High SMA diagnosis rates and accelerated time to treatment ~35,000 Increasing number of patients receiving SMN-targeted SMA patients have 2 therapies for >10 years received an approved SMN-targeted th

6. Slide 6: Poised for 2026 U.S. launch of apitegromab, followed by EMA approval and launch in Europe

   Poised for 2026 U.S. launch of apitegromab, followed by EMA approval and launch in Europe, beginning with Germany UNITED STATES EUROPE EXPANSION to Asia Pacific, LATAM countries Building a 50-country platform to serve patients with rare, severe neuromuscular diseases NOTE: Following regulatory appro

7. Slide 7: Our top priority: Bringing apitegromab to children and adults living with SMA

   Our top priority: Bringing apitegromab to children and adults living with SMA Apitegromab BLA PDUFA Action Date Two Fill-Finish Accepted by FDA of September 30th Facilities Steady and rapid progress U.S. Commercial team is Included in BLA continues in collaboration ready for potential launch resubmi

8. Slide 8: Shaping the future of treatment for patients living with rare neuromuscular disease

   Shaping the future of treatment for patients living with rare neuromuscular disease THERAPEUTIC TARGET INDICATION PRECLINICAL PHASE 1 PHASE 2 PHASE 3 COMMERCIAL Spinal Muscular Atrophy ≥2 Years Spinal Muscular Atrophy <2 Years Selective anti-latent Facioscapulohumeral Muscular myostatin antibody Dys

9. Slide 9: Scholar Rock 2026 priorities

   Scholar Rock 2026 priorities Focused execution and financial discipline 1 2 3 COMMERCIALIZE EXPAND ADVANCE Commercialize apitegromab for Develop apitegromab for patients Advance world-leading treatment of patients with SMA1 with SMA <2 years and for anti-myostatin pipeline additional rare, severe NM

10. Slide 10: Commercialize Apitegromab for Treatment of Patients with SMA

    Commercialize Apitegromab for Treatment of Patients with SMA Transforming the treatment of SMA with muscle-targeted therapy

11. Slide 11: SMA causes motor neuron loss leading to muscle atrophy and progressive weakness

    SMA causes motor neuron loss leading to muscle atrophy and progressive weakness HALLMARKS OF SMA SMN-Targeted Muscle-Targeted Therapies Treatment Spinal Muscular Slow further degeneration of Apitegromab has the potential to Atrophy motor neurons1 directly target progressive muscle ...but do not targ

12. Slide 12: Addressing progressive muscle weakness: Apitegromab positioned to be future standard-of-care

    Addressing progressive muscle weakness: Apitegromab positioned to be future standard-of-care with ongoing SMN-targeted therapy GLOBAL SMA OPPORTUNITY WORLDWIDE IN THE U.S. 90% of patients with SMA rate muscle strength and motor function as top unmet needs5 ~35,000 ~7,000 74% patients have received a

13. Slide 13: Apitegromab successful in Phase 3 SMA study

    Apitegromab successful in Phase 3 SMA study First and only myostatin inhibitor with a positive, statistically significant Phase 3 outcome Phase 3 randomized, placebo-controlled study in SMA patients (N=188) on standard-of-care (nusinersen or risdiplam) 2 HFMSE Total Score by Visit (MITT Set) in Pati

14. Slide 14: Statistically significant, clinically meaningful benefits underscore apitegromab's potential

    Statistically significant, clinically meaningful benefits underscore apitegromab’s potential to impact broad SMA patient population +1.8 HFMSE points2 (p=0.0192) 30.0% vs 12.5% vs. SMN2-targeted treatment alone 30.0% of apitegromab patients Consistent clinically meaningful benefit ACHIEVED ≥3PT acro

15. Slide 15: Two independent paths to potential apitegromab FDA approval by September 30th PDUFA date

    Two independent paths to potential apitegromab FDA approval by September 30th PDUFA date Within 90 days Early Q1 2026 Early Q2 2026 of end of reinspection, FDA/CATALENT FDA COMPLETES per FDA guidelines INDIANA MEETING REINSPECTION FDA CLASSIFIES FACILITY APITEGROMAB CATALENT INDIANA FACILITY POTENTI

16. Slide 16: Muscle strength and motor function remain the top unmet need in children and adults

    Muscle strength and motor function remain the top unmet need in children and adults living with SMA Scholar Rock’s disease education program continues to focus on a broader understanding of SMA 95% O F PAT I E N T S continue to experience persistent and progressive muscle atrophy that limits functio

17. Slide 17: U.S. commercial team operating with urgency to prepare for launch

    U.S. commercial team operating with urgency to prepare for launch TARGETING TARGETING SMA Treatment Centers, SMA Prescribing Physicians, Multi- 140 >2,600 SMA SMA Disciplinary Care Teams Centers Prescribers Patient Engagement & Community Activation National & Regional Payers Medicare & Medicaid SMA,

18. Slide 18: Strong momentum with apitegromab launch readiness in Europe

    Strong momentum with apitegromab launch readiness in Europe in advance of mid-2026 CHMP opinion 1 Building world-class team 2 Engaging the SMA community 3 Establishing access CHMP, Committee for Medicinal Products for Human Use; SMA, Spinal Muscular Atrophy. © 2026 Scholar Rock, Inc. All rights rese

19. Slide 19: Apitegromab in SMA represents a large global opportunity to serve patients

    Apitegromab in SMA represents a large global opportunity to serve patients >$5B 1 1st & Only $2B+ 2 2025 global revenue muscle-targeted Apitegromab for SMN-targeted treatment to show global revenue therapies clinical benefit in SMA potential SMA community is demanding the first and only muscle-targe

20. Slide 20: Develop Apitegromab for Patients with SMA Under 2 and for Additional NMDs

    Develop Apitegromab for Patients with SMA Under 2 and for Additional Neuromuscular Diseases (NMDs) Building a pipeline-in-a-product to reach more patients with SMA and with additional rare, severe NMDs

21. Slide 21: Furthering our commitment to broad SMA community

    Furthering our commitment to broad SMA community Ongoing Phase 2 OPAL study evaluating apitegromab in infants and toddlers with SMA Addressing the needs of children under 2 years of age with SMA to reach patients earlier Expanding our potential impact including evaluation of apitegromab in patients

22. Slide 22: FSHD: Rare, devastating NMD with significant unmet need

    FSHD: Rare, devastating NMD with significant unmet need >30,000 patients diagnosed in U.S. and Europe; no approved therapies FSHD results in progressive muscle atrophy Support for apitegromab therapeutic hypothesis in FSHD leading to cumulative loss of function and Randomized studies of exercise pro

23. Slide 23: Preclinical studies provide mechanistic rationale for apitegromab in FSHD

    Preclinical studies provide mechanistic rationale for apitegromab in FSHD Increased muscle mass, strength, and endurance in gold standard FLExDUX4 mouse model of FSHD FSHD FLEXDUX4 MOUSE MODEL Robust Increase in Significant Improvements in Consistent Gains in MUSCLE MASS MUSCLE FORCE ENDURANCE (28 D

24. Slide 24: Phase 2 trial evaluating apitegromab in patients with FSHD

    Phase 2 trial evaluating apitegromab in patients with FSHD Randomized, double-blind, placebo-controlled, multicenter study (N≈60) SCREENING TREATMENT PRIMARY ENDPOINT: Mean lean muscle volume (LMV) change 4 Weeks 52 Weeks from baseline at 12 months KEY ELIGIBILITY CRITERIA SECONDARY & OTHER ENDPOINT

25. Slide 25: Unlocking pipeline-in-a-product with apitegromab in additional rare NMDs, beginning with FSHD

    Unlocking pipeline-in-a-product with apitegromab in additional rare NMDs, beginning with FSHD Broad landscape of potential indications supports significant opportunity for muscle-targeted therapies GENETIC MYOPATHIES ACQUIRED MYOPATHIES GENETIC NEUROPATHIC Muscular Dystrophies: Inflammatory Myopathi

26. Slide 26: Advance World-Leading Anti-Myostatin Pipeline

    Advance World- Leading Anti-Myostatin Pipeline Driving continued innovation for treatment of patients living with rare, severe NMDs NMDs, neuromuscular diseases. © 2026 Scholar Rock, Inc. All rights reserved.

27. Slide 27: Subcutaneous apitegromab demonstrated favorable bioavailability, with PD profile comparable to IV

    Subcutaneous apitegromab demonstrated favorable bioavailability, with pharmacodynamic profile comparable to IV administration Phase 1 study Overlapping Pharmacodynamic Profiles for IV and SC Apitegromab (800mg) Phase 1 study comparing intravenous (IV) and subcutaneous (SC) 1200 Apitegromab 800 mg IV

28. Slide 28: Advancing Scholar Rock's innovative anti-myostatin platform with SRK-439

    Advancing Scholar Rock’s innovative anti-myostatin platform with SRK-439 Leveraging world-leading expertise to drive continued innovation Latent Myostatin Potency Assay Change in Whole Body Lean Mass Novel, highly potent myostatin Comparing SRK-015P and SRK-439 from Baseline (NHP) inhibitor Inhibiti

29. Slide 29: SRK-439 Phase 1 study assessing safety, tolerability, and PK/PD profile

    SRK-439 Phase 1 study assessing safety, tolerability, and PK/PD profile N≈76 Healthy Adult Participants SRK-439 Program Key Milestones Single Ascending Dose SRK-439 IND cleared in November 2025 PART A Cohorts 1 - 5 R Placebo Dosing commenced in Phase 1 study in December 2025 Multiple Ascending Dose

30. Slide 30: Financials and Upcoming Milestones

    Financials and Upcoming Milestones

31. Slide 31: Operating with financial discipline to achieve our ambitions

    Operating with financial discipline to achieve our ambitions Prioritized investments focused on: Apitegromab commercial launch readiness 1 in the U.S. and Europe $480M Strengthening supply chain to support expanding in cash 2 pipeline and anticipated growing global commercial and equivalents demand

32. Slide 32: Scholar Rock 2026 priorities

    Scholar Rock 2026 priorities Poised to be next global biotech powerhouse 1 2 3 COMMERCIALIZE EXPAND ADVANCE Commercialize apitegromab for Develop apitegromab for patients Advance world-leading treatment of patients with SMA1 with SMA <2 years and for anti-myostatin pipeline additional rare, severe N

33. Slide 33: For more information please contact

    FOR MORE INFORMATION PLEASE CONTACT ir@scholarrock.com media@scholarrock.com www.scholarrock.com © 2026 Scholar Rock, Inc. All rights reserved. 33

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