Form 8-K

8-K — Scholar Rock Holding Corp

Accession: 0001104659-26-056655

Filed: 2026-05-07

Period: 2026-05-07

CIK: 0001727196

SIC: 2836 (BIOLOGICAL PRODUCTS (NO DIAGNOSTIC SUBSTANCES))

Item: Results of Operations and Financial Condition

Item: Regulation FD Disclosure

Item: Financial Statements and Exhibits

Documents

8-K — srrk-20260507x8k.htm (Primary)

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8-K

8-K (Primary)

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Scholar Rock Holding Corporation_May 7, 2026

0001727196false00017271962026-05-072026-05-07

UNITED STATES

SECURITIES AND EXCHANGE COMMISSION

Washington, D.C. 20549

FORM 8-K

CURRENT REPORT

Pursuant to Section 13 or 15(d) of the Securities Exchange Act of 1934

Date of Report (Date of earliest event Reported): May 7, 2026

Scholar Rock Holding Corporation

(Exact Name of Registrant as Specified in Charter)

Delaware

001-38501

82-3750435

(State or Other Jurisdiction of

Incorporation)

(Commission File Number)

(I.R.S. Employer Identification Number)

301 Binney Street, 3rd Floor, Cambridge, MA 02142

(Address of Principal Executive Offices) (Zip Code)

(857) 259-3860

(Registrant’s telephone number, including area code)

(Former name or former address, if changed since last report)

Check the appropriate box below if the Form 8-K filing is intended to simultaneously satisfy the filing obligation of the registrant under any of the following provisions:

☐

Written communications pursuant to Rule 425 under the Securities Act (17 CFR 230.425)

☐

Soliciting material pursuant to Rule 14a-12 under the Exchange Act (17 CFR 240.14a-12)

☐

Pre-commencement communications pursuant to Rule 14d-2(b) under the Exchange Act (17 CFR 240.14d-2(b))

☐

Pre-commencement communications pursuant to Rule 13e-4(c) under the Exchange Act (17 CFR 240.13e-4(c))

Securities registered pursuant to Section 12(b) of the Act:

Title of each class

Trading Symbol(s)

Name of each exchange on which registered

Common Stock, par value $0.001 per share

SRRK

The Nasdaq Global Select Market

Indicate by check mark whether the registrant is an emerging growth company as defined in Rule 405 of the Securities Act of 1933 (17 CFR §230.405) or Rule 12b-2 of the Securities Exchange Act of 1934 (17 CFR §240.12b-2). Emerging growth company ☐

If an emerging growth company, indicate by check mark if the registrant has elected not to use the extended transition period for complying with any new or revised financial accounting standards provided pursuant to Section 13(a) of the Exchange Act. ☐

Item 2.02. Results of Operations and Financial Condition.

On May 7, 2026, Scholar Rock Holding Corporation (the “Company”) issued a press release announcing its financial and operating results for the quarter ended March 31, 2026. A copy of the press release is being furnished as Exhibit 99.1 to this Report on Form 8-K.

The information in this Report on Form 8-K and Exhibit 99.1 attached hereto is intended to be furnished and shall not be deemed “filed” for purposes of Section 18 of the Securities Exchange Act of 1934, as amended (the “Exchange Act”), or otherwise subject to the liabilities of that section, nor shall it be deemed incorporated by reference in any filing under the Securities Act of 1933, as amended, or the Exchange Act, except as expressly set forth by specific reference in such filing.

Item 7.01. Regulation FD Disclosure.

The Company will utilize slides during its conference call scheduled for 8:00 am ET on May 7, 2026 to announce its financial and operating results for the fiscal quarter ended March 31, 2026 and to provide a business update. A copy of the slides is furnished as Exhibit 99.2 to this Current Report on Form 8-K and is incorporated by reference into this Item 7.01 as if fully set forth herein.

The information in this Current Report on Form 8-K and Exhibits 99.1 and 99.2 attached hereto are intended to be furnished and shall not be deemed “filed” for purposes of Section 18 of the Exchange Act, or otherwise subject to the liabilities of that section, nor shall they be deemed incorporated by reference in any filing under the Securities Act or the Exchange Act, except as expressly set forth by specific reference in such filing.

Item 9.01. Financial Statements and Exhibits.

(d) Exhibits

Exhibit

No.

Description

99.1

Press Release issued by the Company on May 7, 2026, furnished hereto.

99.2

Scholar Rock Holding Corporation Corporate Presentation dated May 7, 2026, furnished hereto.

104

Cover Page Interactive Data File (embedded within the Inline XBRL document).

SIGNATURE

Pursuant to the requirements of the Securities Exchange Act of 1934, the registrant has duly caused this report to be signed on its behalf by the undersigned hereunto duly authorized.

Scholar Rock Holding Corporation

Date: May 7, 2026

By:

/s/ Junlin Ho

Junlin Ho

General Counsel & Corporate Secretary

EX-99.1

Filename: srrk-20260507xex99d1.htm · Sequence: 2

Exhibit 99.1

Scholar Rock Reports First Quarter 2026 Financial Results and Recent Business Highlights

• FDA accepted apitegromab Biologics License Application (BLA) for treatment of children and

adults with spinal muscular atrophy (SMA) with September 30, 2026 Prescription Drug User Fee

Act (PDUFA) action date

• Accepted apitegromab BLA includes two fill-finish facilities, Catalent Indiana LLC (part of Novo

Nordisk), and a second U.S.-based facility

• FDA has completed reinspection of Catalent Indiana; classification of facility expected within 90

days following reinspection, in accordance with FDA guidelines

• Second fill-finish facility on track to have commercial apitegromab supply in early Q3 2026

• Scholar Rock is prepared for U.S. apitegromab launch immediately upon FDA approval, which

may be granted at any time through September 30, 2026

• Cash, cash equivalents, and marketable securities of $480 million as of March 31, 2026; includes

an additional $100 million in debt and $98 million in net cash proceeds from the Company’s at-the-market (ATM) program

• Management to host a conference call today at 8:00 a.m. ET

CAMBRIDGE, Mass.--(BUSINESS WIRE)— May 7, 2026 — Scholar Rock (NASDAQ: SRRK), a global

biopharmaceutical company dedicated to improving the lives of children and adults with spinal muscular

atrophy (SMA) and additional rare, severe, and debilitating neuromuscular diseases by applying its

leading platform in myostatin biology to advance musculoskeletal health, today reported financial

results for the first quarter ended March 31, 2026, and provided an update on recent company

developments.

“With the FDA’s acceptance of our apitegromab BLA, we have achieved another critical milestone as we

work with urgency to deliver on our mission to bring the world’s first muscle-targeted treatment to the

SMA community,” said David L. Hallal, Chairman and Chief Executive Officer of Scholar Rock. “We are

grateful for the FDA’s continued high level of engagement, and we are pleased that important progress

continues to be made at both of our fill-finish facilities. Our U.S. commercial team stands ready to

launch apitegromab on or at any time prior to the September 30th PDUFA date.”

Mr. Hallal continued, “Our balance sheet is strong, our clinical-stage pipeline continues to advance, and

we are poised, now more than ever, to usher in the next phase of innovation for patients with SMA.”

Business Highlights and Upcoming Milestones

Apitegromab

Apitegromab is an investigational fully human monoclonal antibody designed to inhibit myostatin

activation by selectively binding the pro- and latent forms of myostatin in skeletal muscle. It is the first

and only muscle-targeted therapeutic candidate in SMA to demonstrate a statistically significant and

clinically meaningful benefit in a pivotal Phase 3 clinical trial (SAPPHIRE).

SMA Program

• Apitegromab BLA accepted by FDA with September 30, 2026 PDUFA action date. The accepted

BLA includes two fill-finish facilities, Catalent Indiana and a second U.S.-based fill-finish facility.

• FDA completed reinspection of Catalent Indiana. Following the FDA’s acceptance of the

apitegromab BLA, the Agency completed reinspection of Catalent Indiana. In accordance with

FDA guidelines, classification of the facility is anticipated within 90 days following reinspection.

• Significant progress continues at second fill-finish facility. Apitegromab commercial supply

from this facility is expected to be available early in the third quarter of 2026.

• Preparations ongoing for U.S. commercial launch. The Commercial team continues to expand

its reach and engagement with key stakeholders, including a significant presence at the

Muscular Dystrophy Association (MDA) Clinical & Scientific Conference, which was held March 8

– 11, 2026 in Orlando, FL and at the upcoming 2026 Annual SMA Conference, which is being

held June 25 – 28, 2026 in Orlando, FL. The U.S. commercial team is prepared to launch

apitegromab immediately upon FDA approval.

• European Medicines Agency (EMA) regulatory review ongoing. A Committee for Medicinal

Products for Human Use (CHMP) opinion for the apitegromab Marketing Authorisation

Application (MAA) is anticipated near mid-2026. The Scholar Rock team in Europe continues to

engage with key stakeholders on SMA disease awareness and education initiatives, including at

the 5th International Scientific Congress on SMA, which was held March 11 – 14, 2026 in

Budapest, Hungary. The Company is planning for an apitegromab launch in Europe in the second

half of 2026, beginning with Germany.

• Enrollment progressing in Phase 2 OPAL clinical trial. Patients continue to be enrolled and

dosed in the Phase 2 OPAL study (NCT07047144). The trial is evaluating apitegromab in infants

and toddlers with SMA under two years of age who have received an approved SMN1-targeted

gene therapy or who are receiving ongoing treatment with an approved SMN2-targeted

therapy.

• Subcutaneous apitegromab development continues to progress. Scholar Rock is advancing a

subcutaneous formulation of apitegromab intended to provide optionality for patients as a small

volume, self- or caregiver-administered anti-myostatin antibody suitable for an autoinjector. A

Phase 1 study in healthy volunteers has been completed. Further development activities are

ongoing, including anticipated FDA and EMA regulatory engagements following apitegromab

approvals.

FSHD Program

• Phase 2 FORGE trial on track for initiation in mid-2026. Scholar Rock is developing apitegromab

for the treatment of people with facioscapulohumeral muscular dystrophy (FSHD). FSHD is a

rare, progressive neuromuscular disease characterized by muscle atrophy and functional

decline, affecting approximately 30,000 individuals across the U.S. and Europe. Initiation of a

Phase 2 randomized, double-blind, placebo-controlled trial, called FORGE, is expected in mid-2026.

SRK-439

SRK-439 is a novel, investigational, subcutaneously administered myostatin inhibitor that binds to pro-and latent myostatin with high affinity and selectivity (i.e., no GDF11 or Activin A binding). Based on

preclinical data, SRK-439 has the potential to potently inhibit myostatin and increase muscle mass.

• Phase 1 healthy volunteer study ongoing. A Phase 1 study evaluating SRK-439 in healthy

volunteers is underway, with topline data expected in the second half of 2026.

First Quarter 2026 Financial Results

Scholar Rock reported a net loss of $105.5 million, including stock-based compensation of $18.2 million,

for the quarter ended March 31, 2026, compared to a net loss of $74.7 million, including stock-based

compensation of $13.4 million, for the quarter ended March 31, 2025. Net loss per common share was

$0.83 for the quarter ended March 31, 2026, compared to $0.67 per common share for the quarter

ended March 31, 2025.

• The Company did not record any revenue for the quarters ended March 31, 2026 and 2025.

• Research and development expense was $51.8 million, including $6.5 million in stock-based

compensation, for the quarter ended March 31, 2026, compared to $48.7 million, including $4.0

million in stock-based compensation, for the quarter ended March 31, 2025.

• General and administrative expense was $50.2 million, including $11.7 million in stock-based

compensation, for the quarter ended March 31, 2026, compared to $28.4 million, including $9.4

million in stock-based compensation, for the quarter ended March 31, 2025.

• As of March 31, 2026, Scholar Rock had cash, cash equivalents, and marketable securities of

$479.9 million. This reflects a drawdown of $100.0 million from the Company’s debt facility and

net cash proceeds of $98.0 million from the Company’s at-the-market (ATM) program.

Conference Call Information

Scholar Rock will host a conference call and webcast today, Thursday, May 7, at 8:00 a.m. ET to review

its first quarter 2026 financial results and discuss recent business updates. To access the live audio

webcast, please go to “Events and Presentations” in the Investors section of the Scholar Rock website at

http://investors.scholarrock.com.

To participate via telephone, please register in advance here. Upon registration, all telephone

participants will receive a confirmation email detailing how to join the conference call.

A replay of the webcast will be available on the Company’s website for approximately 90 days.

About Scholar Rock

Scholar Rock is a late-stage biopharmaceutical company focused on developing and commercializing

apitegromab for children and adults with spinal muscular atrophy (SMA) and other rare, severe and

debilitating neuromuscular diseases. As a global leader in myostatin biology, a field focused on proteins

that regulate muscle mass, the biopharmaceutical company is named for the visual resemblance of a

scholar rock to protein structures. Our commitment to unlock fundamentally different treatment

approaches is powered by broad application of a proprietary platform, which has developed novel

monoclonal antibodies to modulate protein growth factors with extraordinary selectivity. Scholar Rock

works every day to create new possibilities for patients through its highly innovative anti-myostatin

program, including opportunities in additional rare neuromuscular diseases. Learn more at

ScholarRock.com and follow @ScholarRock on X and on LinkedIn.

Scholar Rock® is a registered trademark of Scholar Rock, Inc.

Availability of Other Information About Scholar Rock

Investors and others should note that we communicate with our investors and the public using our

company website www.scholarrock.com, including, but not limited to, company disclosures, investor

presentations and FAQs, Securities and Exchange Commission filings, press releases, public conference

call transcripts and webcast transcripts, as well as on X (formerly known as Twitter) and LinkedIn. The

information that we post on our website or on X or LinkedIn could be deemed to be material

information. As a result, we encourage investors, the media and others interested to review the

information that we post there on a regular basis. The contents of our website or social media shall not

be deemed incorporated by reference in any filing under the Securities Act of 1933, as amended.

Forward-Looking Statements

This press release contains "forward-looking statements" within the meaning of the Private Securities

Litigation Reform Act of 1995, including, but not limited to, statements regarding Scholar Rock’s future

expectations, plans and prospects, including without limitation, Scholar Rock’s expectations regarding its

growth, strategy, progress and timing of its clinical trials and development programs for apitegromab,

including its subcutaneous formulation, SRK-439 and its preclinical programs, and indication selection

and development timing, including the timing of any regulatory submissions, decisions and anticipated

approvals, the therapeutic potential, clinical benefits and safety of any product candidates, its ability to

address the observations identified in the complete response letter, expectations regarding actions by

the FDA after its reinspection of the Catalent Indiana facility; the expected timing and outcome of FDA

review of the accepted BLA for apitegromab, including the September 30, 2026 PDUFA action date;

expectations regarding the availability and timing of commercial supply of apitegromab from Catalent

Indiana and a second U.S.-based fill-finish facility, including expected supply from the second fill-finish

facility; expectations regarding commercial launch timing, and the achievement of important

milestones, the ability of any product candidate to perform in humans in a manner consistent with

earlier nonclinical, preclinical or clinical trial data, the potential of its product candidates and proprietary

platform. The use of words such as “may,” “might,” “could,” “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. 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, whether preclinical and clinical data, including the results from the Phase 3 SAPPHIRE

trial and any results from ongoing or future clinical trials, including the Phase 2 OPAL clinical trial, the

Phase 2 FORGE trial and the Phase 1 clinical trial of SRK-439, will be sufficient to support regulatory

approval or further development; that preclinical and clinical data, including the results from the Phase

2 or Phase 3 clinical trial of apitegromab, data from any ongoing or future trials of apitegromab or data

for SRK-439, are not predictive of, may be inconsistent with, or more favorable than, data generated

from future or ongoing clinical trials of the same product candidates; whether the FDA will accept the

remediations to the Catalent Indiana fill finish facility in response to the FDA Observations, whether the

updated BLA will be sufficient to support regulatory approval, Scholar Rock’s ability to manage expenses

or provide the financial support, resources and expertise necessary to identify and develop product

candidates on the expected timeline; 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; and Scholar Rock’s dependence on third parties

for development and manufacture of product candidates including, without limitation, to supply any

clinical trials 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 March 31, 2026, 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.

2026 2025

Operating expenses

Research and development $ 51,814 $ 48,678

General and administrative 50,202 28,412

Total operating expenses 102,016 77,090

Loss from operations (102,016) (77,090)

Other income (expense), net (3,494) 2,367

Net loss $ (105,510) $ (74,723)

$ (0.83) $ (0.67)

127,277,144 111,838,272

Net loss per share, basic and diluted

Three Months Ended March 31,

Scholar Rock Holding Corporation

Condensed Consolidated Statements of Operations

(unaudited)

(in thousands, except share and per share data)

Weighted average common shares outstanding,

basic and diluted

Investor Contact

Laura Ekas, Ph.D.

ir@scholarrock.com

917-439-0374

Media Contact

Molly MacLeod, Ph.D.

media@scholarrock.com

802-579-5995

March 31, 2026 December 31, 2025

Cash, cash equivalents and

marketable securities $ 479,944 $ 367,563

Other current assets 35,340 17,584

Total current assets 515,284 385,147

Other assets 19,970 19,125

$ 535,254 $ 404,272

Current liabilities $ 59,643 $ 55,419

Long-term liabilities 199,598 103,365

Total liabilities 259,241 158,784

Total stockholders' equity 276,013 245,488

$ 535,254 $ 404,272

Assets

Scholar Rock Holding Corporation

Condensed Consolidated Balance Sheets

(unaudited)

Total liabilities and stockholders' equity

Total assets

Liabilities and Stockholders' Equity

(in thousands)

EX-99.2

Filename: srrk-20260507xex99d2.htm · Sequence: 3

Exhibit 99.2

Q1 2026 Earnings Call

MAY 7, 2026

Exhibit 99.2

TOPIC SPEAKER

BUSINESS UPDATE David L. Hallal

Chairman & Chief Executive Officer

R&D PROGRESS Akshay Vaishnaw, M.D., Ph.D.

President of R&D

COMMERCIAL READINESS Keith Woods

Chief Operating Officer

COMPANY FINANCIALS Vikas Sinha

Chief Financial Officer

5 Q&A SESSION

Q1 2026

EARNINGS

CALL

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, strategy, progress and timing of its clinical trials and development programs

for apitegromab, including its subcutaneous formulation, SRK-439 and its preclinical programs, and indication selection and development timing, including the timing of any

regulatory submissions, decisions and anticipated approvals, the therapeutic potential, clinical benefits and safety of any product candidates, its ability to address the

observations identified in the complete response letter, expectations regarding actions by the FDA after its reinspection of the Catalent Indiana facility; the expected timing

and outcome of FDA review of the accepted BLA for apitegromab; expectations regarding the availability and timing of commercial supply of apitegromab from third-party

U.S.-based fill-finish facility, including expected supply from the second fill-finish facility; expectations regarding commercial launch timing, and the achievement of

important milestones, 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 or Phase 3 clinical trial of apitegromab, data from any ongoing or future trials of apitegromab or data for SRK-439, are not predictive of, may be inconsistent

with, or more favorable than, data generated from future or ongoing clinical trials of the same product candidates; whether the FDA will accept the remediations to the

Catalent Indiana fill finish facility in response to the FDA Observations, whether the updated BLA will be sufficient to support regulatory approval, Scholar Rock’s ability to

manage expenses or provide the financial support, resources and expertise necessary to identify and develop product candidates on the expected timeline; 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 obtain additional funding when needed to support

its business activities; its ability to receive priority or expedited regulatory review or to obtain regulatory approval of apitegromab; its ability to expand globally and the

anticipated commercial launch in the United States of apitegromab in 2026; 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 March 31, 2026, 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 presentation is as of the date of this presentation, 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-439 are investigational drug candidates under evaluation. Apitegromab 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 and SRK-439 have not been established.

Business Update

David L. Hallal

Chairman and Chief Executive Officer

Scholar Rock is positioned for a pivotal year ahead

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

BLA, Biologics License Application; PDUFA, Prescription Drug User Fee Act.

Included in BLA resubmission,

providing two independent

paths to FDA approval

Two Fill-Finish

Facilities

Apitegromab

BLA Accepted

by FDA

Steady and rapid progress

continues in collaboration

with the FDA

U.S. Commercial team

is ready for launch

immediately upon approval,

which may be granted at any

time through September 30th

PDUFA Action Date

of September 30th

Two independent paths to apitegromab FDA approval by September

30th PDUFA date

BLA, Biologics License Application; PDUFA, Prescription Drug User Fee Act.

U.S. Commercial team is ready to launch apitegromab immediately upon FDA approval

FDA Completes

Reinspection

Drug

Product Vialed

To support

FDA review

FDA/Catalent

Indiana Meeting

Early Q1 2026

Type C Meeting with

FDA

Within 90 days of end of

reinspection, per FDA guidelines

FDA Classifies

Facility

Commercial Apitegromab

Supply Available

Early Q3 2026

CATALENT INDIANA

FACILITY

SECOND FILL-FINISH

FACILITY

Apitegromab

BLA Accepted

Submitted late

March; includes

two fill-finish

facilities

POTENTIAL

FDA

APPROVAL

Early Q2 2026

Early March 2026

SHAPING THE FUTURE OF TREATMENT FOR

PATIENTS LIVING WITH RARE NEUROMUSCULAR DISEASES

INITIATE PHASE 2

STUDY for apitegromab

in facioscapulohumeral

muscular dystrophy

SRK-439 PHASE 1

STUDY topline data

in healthy

volunteers

MID - 2026 H2 2026

APITEGROMAB FOR PATIENTS WITH SMA

Myostatin inhibitor with a successful Phase 3 study

Only muscle-targeted treatment to show a statistically

significant, clinically meaningful benefit in SMA

ON TRACK

BLA resubmission accepted by FDA; PDUFA action

date of September 30th

CHMP opinion anticipated near mid-2026

~35,000 have received an SMN-targeted therapy1-3

$2B+ opportunity to serve patients with SMA alone $480M in cash and cash

equivalents as of

March 31, 2026

Scholar Rock is poised for a transformative year

SMA, Spinal Muscular Atrophy; PDUFA, Prescription Drug User Fee Act; CHMP, Committee for Medicinal Products for Human Use; SC, Subcutaneous. 1. Biogen Q4 2023 Report; 2. Roche Q3 2024

report; 3. Novartis Q4 2024 Report.

PHASE 2 OPAL

STUDY for apitegromab

in infants and toddlers

with SMA

UNDERWAY

SC APITEGROMAB

development

activities

ONGOING

R&D Progress

Akshay Vaishnaw, M.D., Ph.D.

President of R&D

Advancing Scholar Rock’s world-leading anti-myostatin pipeline

*Following FDA and EMA regulatory approval of IV apitegromab; YoA, Years of Age; PDUFA, Prescription Drug User Fee Act; CHMP, Committee for Medicinal Products for Human Use.

Program Indication Milestone

Apitegromab

Spinal Muscular

Atrophy ≥2 YoA

PDUFA action date of

September 30 CHMP opinion mid-2026

Facioscapulohumeral

muscular dystrophy Phase 2 FORGE study initiation in mid-2026

SRK-439 Healthy Volunteers Phase 1 study topline results expected in H2 2026

Subcutaneous

Apitegromab

Development activities ongoing, with anticipated FDA and

EMA regulatory engagements on clinical path forward*

Spinal Muscular

Atrophy

Phase 2 OPAL study in infants

and toddlers ongoing

Spinal Muscular

Atrophy <2 YoA

Evaluating PK, PD, efficacy,

safety, and tolerability of

apitegromab over 48 weeks

1 0

Furthering our commitment to broad SMA community

Ongoing Phase 2 OPAL study evaluating apitegromab in infants and toddlers with SMA

PK, Pharmacokinetic; PD, Pharmacodynamic.

Patient enrollment and dosing underway in Phase 2 OPAL study

including evaluation of apitegromab in patients

who received SMN1-targeted gene therapy

Expanding our potential impact

to reach patients earlier

Focused on addressing the needs of

children <2 years of age with SMA

seeking to address the motor neuron and muscle

in youngest patients

Time is muscle

1 1

FSHD: Rare, devastating NMD with significant unmet need

>30,000 patients diagnosed in U.S. and Europe1-3; no approved therapies

FLExD, FLExDUX4.Cre; FSHD, facioscapulohumeral muscular dystrophy; NMD, Neuromuscular Disease; WT, wild type; Ab, antibody; 6MWD, 6-minute walk distance. 1.Scholar Rock. Data on file.

2. Tihaya MS, et al. Nat Rev Neurol. 2023;19(2):91-108. 3. Attarian S, et al. J Neurol. 2024;271(9):5778-5803. 4. Fogel A. Presented at: FSHD Society International Research Congress; Jun 12-

13, 2025; Amsterdam, Netherlands. 5. Bankole, LC, et al. Medicine. 2016; 95(31). 6. Andersen, G, et al. Neurology. 2015; 85:396-403. 7. Heatwole, CR, et al. Neurol Genet. 2025;11:e200292.

Support for apitegromab therapeutic hypothesis in FSHD

Randomized studies of exercise programs suggest muscle has

capacity to show functional benefit5,6

Study of anabolic agents suggests increase in lean mass and

muscle function7

Increase in Lean Body Mass Improvement in 6MWD

FSHD FLExDUX4 Mouse Model

Robust Increase in Muscle

Mass (28 Days)4

Control Ab

muSRK-015P

TA Weight (mg)

11%

Phase 2 FORGE study on track to initiate mid-2026

1 2

Advancing innovation with subcutaneous apitegromab and SRK-439

SRK-439 is a novel, investigational myostatin inhibitor.

Leveraging world-leading expertise to drive continued innovation

Subcutaneous Apitegromab

Phase 1 study comparing

intravenous (IV) and subcutaneous

(SC) apitegromab in healthy

volunteers

At 800mg, SC and IV apitegromab

produced overlapping PD

responses (total latent myostatin)

Further development activities

ongoing, including anticipated FDA

and EMA regulatory engagements

following apitegromab approvals

SRK-439

Novel, highly potent myostatin

inhibitor

Phase 1 study in healthy

volunteers in progress; topline

data expected in H2 2026

Optimized for subcutaneous

administration

Strong preclinical data

demonstrating favorable muscle

mass preservation

Commercial Readiness

Keith Woods

Chief Operating Officer

1 3

1 4

Muscle strength and motor function remain the top unmet need

in children and adults living with SMA

SMA, Spinal Muscular Atrophy.

Scholar Rock’s disease education

program continues to focus on

a broader understanding of SMA

as a disease of the motor unit—

which consists of both the

motor neuron and the muscle

MUSCLE

MOTOR

NEURON

OF PATIENTS

continue to experience persistent and progressive 95%

muscle atrophy that limits function and independence

OF PEOPLE LIVING WITH SMA

IN THE U.S.

have received two or more FDA approved SMA

treatments, either sequentially or in combination

~1/3

1 5

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

SMA, Spinal Muscular Atrophy.

SMA Treatment Centers, SMA

Prescribing Physicians, Multi-Disciplinary Care Teams

TARGETING

>2,600

SMA

Prescribers

TARGETING

140

SMA

Centers

Patient Engagement &

Community Activation

National & Regional Payers

Medicare & Medicaid

1 6

Strong momentum with apitegromab launch readiness in Europe in

advance of mid-2026 CHMP opinion

SMA, Spinal Muscular Atrophy; CHMP, Committee for Medicinal Products for Human Use.

1 Building world-class team

2 Engaging the SMA community

3 Establishing access

~35,000

SMA patients have received an approved

SMN-targeted therapy1-3

Powering Scholar Rock through the end of this decade and into the next

1 7

Global apitegromab opportunity in SMA alone offers potential

for many years of sustainable growth

1. Biogen Q4 2023 Report; 2. Roche Q3 2024 report; 3. Novartis Q4 2024 Report. SMN, survival motor neuron.

Company Financials

Vikas Sinha

Chief Financial Officer

1 8

1 9

Operating with financial discipline to achieve our ambitions

as of March 31, 2026

$480M

in cash and equivalents

Prioritized investments focused on:

Apitegromab commercial launch readiness

in the U.S. and Europe 1

Strengthening supply chain to support expanding

pipeline and anticipated growing global

commercial demand for apitegromab over time

3 Advancing highly innovative clinical programs

Closing Remarks

David L. Hallal

Chairman and Chief Executive Officer

2 0

Q&A

2 1

For more information, please contact:

ir@scholarrock.com

media@scholarrock.com

Or visit us at www.scholarrock.com

2 2

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