Scholar Rock : Corporate Presentation March 2024

Deep Insights

Advancing

Impactful Medicines

Company Overview | March 2024

© 2024 Scholar Rock, Inc. All rights reserved.

Forward-Looking Statements

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 preclinical studies and clinical trials for SRK-439, 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 or Part B of the Phase 1 trial of SRK-181, are not predictive of, may be inconsistent with, or more favorable than, data generated from future or ongoing 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, 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 Form 10-K for the year ended December 31, 2022, and Quarterly Report on Form 10-Q for the quarter ended September 30, 2023, 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.

This presentation may also contain estimates and other statistical data made by independent parties and by us relating to market size and growth and other data about our industry. This data involves a number of assumptions and limitations, and you are cautioned not to give undue weight to such estimates. In addition, projections, assumptions, and estimates of our future performance and the future performance of the markets in which we compete are necessarily subject to a high degree of uncertainty and risk.

Apitegromab and SRK-181 are investigational drug candidates under evaluation. Apitegromab, SRK-181, and SRK-439 have not been approved for any use by the FDA or any other regulatory agency and the safety and efficacy of apitegromab, SRK-181 and SRK-439 have not been established.

© Scholar Rock, Inc. All rights reserved. 2024.

We are a global leader in harnessing the life-changing potential of TGFβ biology

OUR MISSION

To discover, develop, and deliver life-changing therapies by harnessing cutting-edge science to create new possibilities for people living with serious diseases

TGFβ=Transforming growth factor-beta.

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Building a Fully Integrated Therapeutics Company

Revolutionary

Scientific Platform

• Pioneers in unparalleled
  selective targeting of the latent forms of growth factors
• Robust pipeline of novel assets including two clinical programs and a growing portfolio of preclinical programs

Transformative Therapeutics in Development

Apitegromab in SMA

Potential therapy in Ph 3 designed to improve motor function to help address remaining unmet need after receipt of existing SMA therapies

SRK- 439 in Obesity

Novel antimyostatin antibody with the potential to support healthier weight management by preserving lean muscle

SRK-181 in Immuno-Oncology

In Ph 1 development to overcome resistance to checkpoint inhibitors in

multiple tumor types

Experienced

and Focused

• Seasoned team with track record of clinical and commercial success
• Deep rare disease, R&D, FDA/EMA approval & launch experience
• Focused, efficient approach to scaling the organization

SMA=Spinal muscular atrophy; EMA=European Medicines Agency; FDA=United States Food and Drug Administration; R&D=research and development.

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© 2024 Scholar Rock, Inc. All rights reserved.

Our Approach

S e l e c t i v i t y D r i v e s S u c c e s s

RIGHT	V a l i d a t e d
TARGET	B i o l o g y

RIGHT L a t e n t

TIME F o r m

Deep structural insights to validated targets

Industry-leading antibody design and protein engineering to selectively target latent growth factors

Optimized for efficacy and mitigates off-target effects

Traditional Target	Scholar Rock's Target
"mature" active growth factor	L Latent Growth Factor

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© 2024 Scholar Rock, Inc. All rights reserved.

Advancing a Robust Pipeline with Our Differentiated Platform

TARGET		DISCOVERY/
	PRECLINICAL	PHASE 1	PHASE 2	PHASE 3
	SPINAL MUSCULAR ATROPHY
	Apitegromab

Latent	CARDIOMETABOLIC DISORDERS
Myostatin
Apitegromab in Obesity*

SRK-439 (novel antimyostatin antibody)

	IMMUNO-ONCOLOGY
Latent	SRK-181 (selective context-independent,
anti-latentTGFβ-1)
TGFβ-1	FIBROSIS
	Selective context-dependent
	(LTBP1 & LTBP3) anti-latentTGFβ-1
RGMc	ANEMIA
Selective anti-RGMc

Potential to transform the lives of people living with a wide range of serious diseases, including neuromuscular disorders, cardiometabolic disorders, oncology, and fibrosis

* Subject to receipt of regulatory authority approval. We plan to utilize data from a previously completed Ph 1 study in healthy volunteers and initiate a Ph 2 POC trial in 2024.

LTBP1=Latent transforming growth factor beta binding protein 1; LTBP3=Latent transforming growth factor beta binding protein 3; POC=Proof of concept; RGMc=Repulsive guidance molecule C;	6
TGFβ-1=Transforming growth factor beta-1.

© 2024 Scholar Rock, Inc. All rights reserved.

Antimyostatin Program:

Apitegromab in Spinal Muscular

Atrophy

© 2024 Scholar Rock, Inc. All rights reserved.

Hallmarks of SMA

Motor Neuron Loss and Muscle Atrophy Leads to Progressive Muscle Weakness

S P I N A L M U S C U L A R A T R O P H Y

Spinal Muscular Atrophy

SMN therapies

slow further degeneration of motor neurons1

...but do not directly address muscle atrophy

Motor neuron impairment and loss due to SMN genetic deficiency leads to muscle atrophy and weakness

Muscle fiber atrophy

Motor neuron degeneration2

There is further potential to

regain vital muscle function by also addressing the progressive muscle

atrophy and associated weakness of SMA

SMA=Spinal muscular atrophy; SMN=Survival motor neuron.	8
1. Hua Y, et al. Nature. 2011;478(7367):123-6.
2. Figure adapted from: SMA Foundation Overview. http://www.smafoundation.org/wp-content/uploads/2012/03/SMA-Overview.pdf.; Accessed April 18, 2021.

© 2024 Scholar Rock, Inc. All rights reserved.

S P I N A L M U S C U L A R A T R O P H Y

SMA Leads to Deterioration in Essential Muscle Function

What may seem like minimal gains in strength actually translate to exponential gains in functional abilities.

I often have to choose between taking a shower and doing homework because I don't have the energy to do both.

Small tasks are huge success in my life. If I could lift that 1L bottle of water at work instead of having to find a graduate student to move it for me…things don't take a ton more muscle, but they are all muscle I still don't have.

Despite significant advancements,	Muscle weakness can lead to
progressive muscle weakness	deterioration in mobility, swallowing,
remains an unmet need in SMA	breathing and cause debilitating fatigue

Quotes are from patient advocates who participated in 2022 Cure SMA FDA Patient-Led Listening Session and not from the pictured individuals. Summary of the listening session can be found on	9
the FDA website at https://www.curesma.org/cure-sma-holds-patient-led-listening-session-with-fda/

© 2024 Scholar Rock, Inc. All rights reserved.

S P I N A L M U S C U L A R A T R O P H Y

SMA Today: More Patients Screened and Treated

GLOBAL DISEASE:

>20,000 affected

in US and Europe1, 2

Three treatments to address SMN loss

>13,000 patients	> 11,000 patients	> 3,500 patients
treated WW	treated WW	treated WW
$1.8 billion	~CHF1.4 billion	~$1.2 billion
annual revenue (LTM)3	annual revenue (LTM)4	in revenues (LTM)5

Established market dynamics support Scholar Rock's first potential commercial launch

CHF=Swiss franc; LTM=last twelve months; SMA=Spinal muscular atrophy; SMN=Survival motor neuron; WW=worldwide.
1. Cure SMA 2022 Report: 9042022_State-of-SMA_vweb.pdf(curesma.org)
2.	Lally et al. Indirect estimation of the prevalence of spinal muscular atrophy Type I, II, and III in the United States. Orphanet J Rare Dis. 2017 Nov 28;12(1):175. doi: 10.1186/s13023-017-0724-z.
3.	Revenue as of Biogen 3Q23 financial update; includes patients treated worldwide in post-marketing setting, expanded access program, and clinical trials as of May 2022.
4. Revenue as of Roche 3Q23 financial update; includes patients treated worldwide as of July 2023.	10
5.	Revenue as of Novartis 3Q23 financial update; includes patients treated worldwide including clinical trials, commercially, and managed access programs as of August 2023.

© 2024 Scholar Rock, Inc. All rights reserved.