Scholar Rock : Corporate Presentation January 2023

Deep Insights

Advancing

Impactful Medicines

January 2023

© 2022 Scholar Rock, Inc. All rights reserved.

Disclaimers

Various statements in this presentation concerning the future expectations, plans and prospects of Scholar Rock, Inc. ("Scholar Rock"), including without limitation, Scholar Rock's expectations regarding its strategy, its product candidate selection and development timing, including timing for the initiation of and reporting results from its clinical trials for apitegromab, SRK-181, and other product candidates and indication selection and development timing, its cash runway,

the ability of any product candidate to perform in humans in a manner consistent with earlier nonclinical, preclinical or clinical trial data, and the potential of its product candidates and proprietary platform. The use of words such as "may," "could," "might," "will," "should," "expect," "plan," "anticipate," "believe," "estimate," "project," "intend," "future," "potential," or "continue," and other similar expressions are intended to identify such forward-looking statements for the purposes of the safe harbor provisions under The Private Securities Litigation Reform Act of 1995. 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. These risks and uncertainties include, without limitation, that preclinical and clinical data, including the results from the Phase 2 trial of apitegromab or Part A of the Phase 1 trial of SRK-181, are not predictive of, may be inconsistent with, or more favorable than, data generated from future clinical trials of the same product candidate, including the Phase 3 clinical trial of apitegromab in SMA and Part B of the Phase 1

clinical trial of SRK-181, respectively, Scholar Rock's ability to provide the financial support, resources and expertise necessary to identify and develop product

candidates on the expected timeline, the data generated from Scholar Rock's nonclinical and preclinical studies and clinical trials, information provided or decisions made by regulatory authorities, competition from third parties that are developing products for similar uses, Scholar Rock's ability to obtain, maintain and protect its intellectual property, the success of Scholar Rock's current and potential future collaborations, Scholar Rock's dependence on third parties for development and manufacture of product candidates including, without limitation, to supply any clinical trials, Scholar Rock's ability to manage expenses and to obtain additional funding when needed to support its business activities and establish and maintain strategic business alliances and new business initiatives, and the impacts of current macroeconomic and geopolitical events, including changing conditions from the COVID-19 pandemic, hostilities in Ukraine, increasing rates of inflation and rising interest rates, on business operations and expectations, as well as those risks more fully discussed in the section entitled "Risk Factors" in Scholar Rock's Quarterly Report on Form 10-Q for the quarter ended September 30, 2022, as well as discussions of potential risks, uncertainties, and other important factors in Scholar Rock's subsequent filings with the Securities and Exchange Commission. Any forward-looking statements represent Scholar Rock's views only as of today and should not be relied upon as representing its views as of any subsequent date. All information in this press release is as of the date of the release, and Scholar Rock undertakes no duty to update this information unless required by law.

Apitegromab and SRK-181 are investigational drug candidates under evaluation. Apitegromab and SRK-181 have not been approved for any use by the FDA or any other regulatory agency and the safety and efficacy of apitegromab and SRK-181 have not been established.

© Scholar Rock, Inc. All rights reserved.

Scholar Rock:

Revolutionary Platform

• Global leader in TGFβ superfamily biology
• Targeting the latent forms of growth factors
• Exquisite selectivity to deliver differentiated therapeutic profiles

Transforming

Patient Lives,

Targeting High

Neuromuscular and Beyond

• Rich preclinical pipeline focused on high unmet patient needs
• Phase 3 SAPPHIRE study underway, data readout expected in 2024
• Phase 1 POC DRAGON study underway in immuno-oncology

Unmet Medical Need

Positioned for Success

• Compelling proof-of-concept TOPAZ data informed Phase 3 SAPPHIRE study design
• Seasoned leadership team with track record of clinical and commercial success
• Anticipated cash runway into 2025

Strategic Optionality

• Commercial planning underway for apitegromab (SMA) in US and Europe
• Broad platform, including promising early-stage assets, provides opportunities to advance alone or in partnership

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© 2022 Scholar Rock, Inc. All rights reserved.

Revolutionary Approach to Regulating TGFβ Superfamily Implicated in Devastating Diseases

Scholar Rock's Target

TGFβ Superfamily: Highly Sought-After Targets

Latent Growth Factor

Recognized by the industry as important targets given their fundamental roles in regulating a variety of cellular processes

Dysregulation plays a role in devastating diseases that have a high unmet need, including:

• Neuromuscular disorders
• Fibrosis
• Oncology

Traditional Target

"mature" growth factor

Scholar Rock's R&D Platform

Transforming Medical Practice

• Selectively target the latent form of growth factors in the microenvironment of cells and tissues with uniquely designed antibodies
• Overcome the challenges that plague traditional approaches that target the "mature" growth factor, which are difficult to differentiate and lead to unintended negative effects

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© 2022 Scholar Rock, Inc. All rights reserved.

Robust Pipeline of Novel Product Candidates

DISCOVERY/
PRECLINICAL	PHASE 1	PHASE 2	PHASE 3	2023 MILESTONES

SPINAL MUSCULAR ATROPHY

Apitegromab (selective anti-latent myostatin)

IMMUNO-ONCOLOGY

SRK-181 (Selective context-independent,anti-latentTGFβ-1)

ANEMIA

Selective anti-RGMc

FIBROSIS

Selective context-dependent (LTBP1 & LTBP3) anti-latentTGFβ-1

36-month TOPAZ data

SAPPHIRE: LPI

Rolling clinical data updates

IND-enabling studies

IND-enabling studies

Potential to transform the lives of patients suffering from a

wide range of serious diseases, including neuromuscular disorders, oncology, and fibrosis

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© 2022 Scholar Rock, Inc. All rights reserved.