Scholar Rock Holding Corporation · NASDAQ:SRRK

Scholar Rock — Investor Presentation (October 2023)

24 slides from Scholar Rock's investor presentation, Oct 11, 2023

Slide 1: Introduction of Scholar Rock's Cardiometabolic Disorders Program
| CONFIDENTIAL AND PROPRIETARY Introduction of Scholar Rock’s Cardiometabolic Disorders Program Optimizing weight loss by preserving and increasing lean muscle mass with a highly selective myostatin inhibitor
Slide 2: Forward-Looking Statements
© Scholar Rock, Inc. All rights reserved. 2023. Forward - Looking Statements Various statements in this presentation concerning the future expectations, plans and prospects of Scholar Rock, Inc. (“Schol ar Rock”), including without limitation, Scholar Rock’s expectations regarding its strategy, its
Slide 3: Scholar Rock: Transforming Patient Lives, Addressing High Unmet Medical Need
© 2023 Scholar Rock, Inc. All rights reserved. Scholar Rock: Transforming Patient Lives, Addressing High Unmet Medical Need 3 • Global leader in TGF β superfamily biology • Targeting the latent forms of growth factors • Exquisite selectivity to deliver differentiated therapies Revolutionary Platform
Slide 4: Revolutionary Approach to Regulating TGFβ Superfamily Implicated in Devastating Diseases
© 2023 Scholar Rock, Inc. All rights reserved. Revolutionary Approach to Regulating TGF β Superfamily Implicated in Devastating Diseases 4 Scholar Rock’s R&D Platform Goals Aiming to Transform Medical Practice • Selectively target the latent form of growth factors in the microenvironment of cells an
Slide 5: Differentiated Expertise Developing Muscle-Targeted Therapies
© 2023 Scholar Rock, Inc. All rights reserved. Differentiated Expertise Developing Muscle - Targeted Therapies 5 Next Frontier in Antimyostatin Leveraging Our R&D Platform to Expand into Cardiometabolic Disorders • Pioneered unique approach to develop antibodies that bind to pro - and latent forms o
Slide 6: Robust Pipeline of Novel Product Candidates
© 2023 Scholar Rock, Inc. All rights reserved. Potential to transform the lives of people living with a wide range of serious diseases, including neuromuscular disorders, cardiometabolic, oncology, and fibrosis TARGET DISCOVERY/ PRECLINICAL PHASE 1 PHASE 2 PHASE 3 ANTICIPATED KEY MILESTONES Latent M
Slide 8: Obesity is a common, serious, and costly chronic disease affecting adults and children worldwide
8 Obesity is a common, serious, and costly chronic disease affecting adults and children worldwide Obesity is Recognized as a Top Global Public Health Issue By 2030, obesity will affect > 1 billion adults and >250 million children and adolescents 1 In the US, 1 in 5 children and more than 1 in 3 adu
Slide 9: Loss of Lean Muscle Is Significant with GLP-1 RA Therapies
9 Loss of Lean Muscle Is Significant with GLP - 1 RA Therapies Lean muscle is essential to healthy metabolic function & body composition Situation • Significant challenges with current weight loss strategies (dietary/behavioral, pharmacological, surgical), including tolerability, lack of durability
Slide 10: Obesity Landscape is Evolving & Growing: Estimated $70-$100B Annual Revenues by 2035
10 Obesity Landscape is Evolving & Growing: Estimated $70 - $100B Annual Revenues by 2035 1 1. Seigerman , ED, et al. “Obesity Exceptionalism: It’s different this time.” BMO Capital Markets: September 2023 retatrutide 2010 2014 2017 2021 2023 2025 2022
Slide 11: Recent KOL Interviews: Select Physicians See Value and Benefit in Muscle Maintenance
11 Recent KOL Interviews: Select Physicians See Value and Benefit in Muscle Maintenance During Weight Loss » Some key opinion leaders (KOLs) see a lack of standard of care (SOC) in obesity which leads to significant unmet need in large patient population » KOLs see the potential for additional benef
Slide 12: Therapeutic Strategy for Safe, Durable Weight Loss Could Include a Highly Selective Antimyostatin
12 We believe Therapeutic Strategy for Safe, Durable Weight Loss Could Include a Highly Selective Antimyostatin to Retain Lean Muscle Opportunity • Inhibition of myostatin has demonstrated important physical (increased muscle mass) and beneficial metabolic effects (insulin sensitivity, basal metabol
Slide 13: Scholar Rock Antibodies Selectively Inhibit Activation of Myostatin
13 Scholar Rock Antibodies Selectively Inhibit Activation of Myostatin Selective Targeting of Proforms of Myostatin SRRK antibodies do not bind to mature myostatin or any form of GDF11, Activin A, or other TGF - β family members Selectivity is critical to avoid safety concerns ▪ Mature myostatin and
Slide 15: SRK-439: New Preclinical Myostatin Inhibitor Candidate
15 SRK - 439: New Preclinical Myostatin Inhibitor Candidate Represents Opportunity to Address Cardiometabolic Patient Population Antibody/ Variant mTLL2 IC 50 ( nM ) mAb K D ( nM ) SRK - 439 0.145 0.579 • SRK - 439 , a novel antimyostatin antibody being developed by SRRK, has attractive properties a
Slide 16: SRK-439 Maintained Lean Mass in Mouse DIO Model When Combined with GLP-1 RA Therapy
16 SRK - 439 Maintained Lean Mass in Mouse Diet Induced Obesity (DIO) Model When Combined with GLP - 1 RA Therapy -80 -60 -40 -20 0 20 40 60 80 % Change Fat Mass from Baseline (qNMR) % C h a n g e IgG IgG 3 1 0.3 0.06mg/kg Lira SRK-439 ▪ Improved fat mass loss vs GLP - 1 RA alone -20 -10 0 10 20 % C
Slide 17: SRK-439 Dose-Dependently Reversed Lean Mass Loss in Mouse DIO with Semaglutide
17 SRK - 439 Dose - Dependently Reversed Lean Mass Loss in Mouse DIO and Enhanced Fat Mass Loss Induced by Semaglutide Treatment -80 -60 -40 -20 0 20 % Change - Fat Mass (qNMR) % C h a n g e IgG S R K - 4 3 9 IgG 0.1 0.3 1.0 3.0 SRK-439 (mg/kg) Semaglutide 0.04 mg/kg -20 -10 0 10 20 % Change - Lean
Slide 18: Selective Myostatin Inhibitors Represent an Opportunity to Address Unmet Need in Cardiometabolic Disorders
18 ▪ Preclinical data suggests mechanism of action that could safely reduce body fat while maintaining muscle , thereby enhancing metabolic health ▪ Studies in DIO mice on the effects of GLP - 1 receptor agonist (GLP - 1 RA) in combination with a myostatin - selective inhibitor showed: – Dose - depe
Slide 19: Plan to Expedite Cardiometabolic Program with Phase 2 Proof of Concept Study of Apitegromab in Obesity
19 Plan to Expedite Cardiometabolic Program with Phase 2 Proof of Concept Study of Apitegromab in Obesity SRK - 439 Rapid Proof of Concept Phase 2 Study with A pitegromab in combination with GLP - 1 APITEGROMAB While a dvanc ing investigational SRK - 439 for cardiometabolic indication 2023 2024 2025
Slide 20: Differentiation, Scientific Rationale and Investment Opportunity
© 2023 Scholar Rock, Inc. All rights reserved. 20 • Preclinical data suggests selective mechanism of action that can maintain muscle mass while enhancing fat mass loss, thereby improving metabolic function • SRK - 439: Preclinical data supports the overall target candidate profile to address the car
Slide 23: Ongoing SAPPHIRE Phase 3 Trial Overview
© 2023 Scholar Rock, Inc. All rights reserved. Ongoing SAPPHIRE Phase 3 Trial Overview 23 Randomized, double - blind, placebo - controlled, parallel arm design (n=204) Enrolling patients who are on SMN - directed therapy ( nusinersen or risdiplam ) Anticipate completing enrollment in 3Q 2023 TREATME
Slide 24: Executing on the Promise: Apitegromab SMA Trials
© 2023 Scholar Rock, Inc. All rights reserved. Executing on the Promise: Apitegromab SMA Trials 24 SMA=Spinal Muscular Atrophy *Subject to regulatory approval WHERE WE ARE Phase 3 12 - Month IN PROCESS Long - term EXTENSION 2023 enrollment completed Q4 2024 data 2025 launch* ANTICIPATED MILESTONES W

Scholar Rock Holding — Investor Presentation (October 2023) on 6ix

Scholar Rock — Investor Presentation (October 2023)

Slide 1: Introduction of Scholar Rock's Cardiometabolic Disorders Program

Slide 2: Forward-Looking Statements

Slide 3: Scholar Rock: Transforming Patient Lives, Addressing High Unmet Medical Need

Slide 4: Revolutionary Approach to Regulating TGFβ Superfamily Implicated in Devastating Diseases

Slide 5: Differentiated Expertise Developing Muscle-Targeted Therapies

Slide 6: Robust Pipeline of Novel Product Candidates

Slide 7: Next Horizon: Cardiometabolic Disorders

Slide 8: Obesity is a common, serious, and costly chronic disease affecting adults and children worldwide

Slide 9: Loss of Lean Muscle Is Significant with GLP-1 RA Therapies

Slide 10: Obesity Landscape is Evolving & Growing: Estimated $70-$100B Annual Revenues by 2035

Slide 11: Recent KOL Interviews: Select Physicians See Value and Benefit in Muscle Maintenance

Slide 12: Therapeutic Strategy for Safe, Durable Weight Loss Could Include a Highly Selective Antimyostatin

Slide 13: Scholar Rock Antibodies Selectively Inhibit Activation of Myostatin

Slide 14: SRK-439 Preclinical Data

Slide 15: SRK-439: New Preclinical Myostatin Inhibitor Candidate

Slide 16: SRK-439 Maintained Lean Mass in Mouse DIO Model When Combined with GLP-1 RA Therapy

Slide 17: SRK-439 Dose-Dependently Reversed Lean Mass Loss in Mouse DIO with Semaglutide

Slide 18: Selective Myostatin Inhibitors Represent an Opportunity to Address Unmet Need in Cardiometabolic Disorders

Slide 19: Plan to Expedite Cardiometabolic Program with Phase 2 Proof of Concept Study of Apitegromab in Obesity

Slide 20: Differentiation, Scientific Rationale and Investment Opportunity

Slide 21: Thank You

Slide 22: Appendix

Slide 23: Ongoing SAPPHIRE Phase 3 Trial Overview

Slide 24: Executing on the Promise: Apitegromab SMA Trials