A Study to Evaluate How Apitegromab Works in Subjects Who Are Less Than 2 Years Old and Have Spinal Muscular Atrophy

NCT ID: NCT07047144

Last Updated: 2026-01-26

Study Results

Results pending

The study team has not published outcome measurements, participant flow, or safety data for this trial yet. Check back later for updates.

Basic Information

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Recruitment Status

RECRUITING

Clinical Phase

PHASE2

Total Enrollment

52 participants

Study Classification

INTERVENTIONAL

Study Start Date

2025-09-15

Study Completion Date

2029-03-31

Brief Summary

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This double-blind, Phase 2, multiple-dose study will be conducted to evaluate the PK/PD, efficacy, safety, and tolerability of apitegromab in subjects \<2 years old with 5q autosomal recessive SMA who have delayed motor milestones for their age attributed to SMA at the discretion of the Investigator or a Children's Hospital of Philadelphia Infant Test of Neuromuscular Disorders (CHOP-INTEND) score \<55.

Detailed Description

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Conditions

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Spinal Muscular Atrophy SMA Spinal Muscular Atrophy Type 2 Spinal Muscular Atrophy Type 3 Neuromuscular Manifestations Anti-myostatin

Study Design

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Allocation Method

RANDOMIZED

Intervention Model

PARALLEL

Primary Study Purpose

TREATMENT

Blinding Strategy

QUADRUPLE

Participants Caregivers Investigators Outcome Assessors
The Sponsor, the subject's caregiver, Investigators, and site personnel, with the exception of the designated unblinded personnel (eg, site Pharmacist), will be blinded to the treatment assignment.

Study Groups

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Apitegromab low dose + SMN Therapy

Patients who are less than 2 years of age with SMA will receive apitegromab every 4 weeks by intravenous (IV) infusion during the 48-week Treatment Period.

Patients must have been treated with an approved SMN1-targeted therapy (ie, onasemnogene abeparvovec-xioi) or are continuing to be treated with an approved SMN2-targeted therapy (ie, nusinersen or risdiplam).

Group Type EXPERIMENTAL

Apitegromab

Intervention Type DRUG

Apitegromab is a fully human anti-proMyostatin monoclonal antibody (mAb) of the immunoglobulin G4 (IgG4)/lambda isotype that specifically binds to human pro/latent myostatin with high affinity inhibiting myostatin activation. SRK-015 will be administered every 4 weeks by intravenous (IV) infusion.

Nusinersen

Intervention Type DRUG

Nusinersen is a current standard-of-care SMN therapy that targets the SMN2 gene. It will be administered intrathecally per the prescribing information.

Risdiplam

Intervention Type DRUG

Risdiplam is a current standard-of-care SMN therapy that targets the SMN2 gene. It will be administered orally per the prescribing information.

Apitegromab high dose + SMN Therapy

Patients who are less than 2 years of age with SMA will receive apitegromab every 4 weeks by intravenous (IV) infusion during the 48-week Treatment Period.

Patients must have been treated with an approved SMN1-targeted therapy (ie, onasemnogene abeparvovec-xioi) or are continuing to be treated with an approved SMN2-targeted therapy (ie, nusinersen or risdiplam).

Group Type EXPERIMENTAL

Apitegromab

Intervention Type DRUG

Apitegromab is a fully human anti-proMyostatin monoclonal antibody (mAb) of the immunoglobulin G4 (IgG4)/lambda isotype that specifically binds to human pro/latent myostatin with high affinity inhibiting myostatin activation. SRK-015 will be administered every 4 weeks by intravenous (IV) infusion.

Nusinersen

Intervention Type DRUG

Nusinersen is a current standard-of-care SMN therapy that targets the SMN2 gene. It will be administered intrathecally per the prescribing information.

Risdiplam

Intervention Type DRUG

Risdiplam is a current standard-of-care SMN therapy that targets the SMN2 gene. It will be administered orally per the prescribing information.

Interventions

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Apitegromab

Apitegromab is a fully human anti-proMyostatin monoclonal antibody (mAb) of the immunoglobulin G4 (IgG4)/lambda isotype that specifically binds to human pro/latent myostatin with high affinity inhibiting myostatin activation. SRK-015 will be administered every 4 weeks by intravenous (IV) infusion.

Intervention Type DRUG

Nusinersen

Nusinersen is a current standard-of-care SMN therapy that targets the SMN2 gene. It will be administered intrathecally per the prescribing information.

Intervention Type DRUG

Risdiplam

Risdiplam is a current standard-of-care SMN therapy that targets the SMN2 gene. It will be administered orally per the prescribing information.

Intervention Type DRUG

Other Intervention Names

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SRK-015 Spinraza Evrysdi

Eligibility Criteria

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Inclusion Criteria

1. Is \<2 years old at the time of the informed consent
2. Had a gestational age of ≥35 weeks and gestational body weight ≥2.0 kg at birth
3. Has confirmed diagnosis of 5q autosomal recessive SMA
4. Has confirmed presence of SMN2 gene copy(ies)
5. Must have been treated with an approved SMN1-targeted therapy (ie, onasemnogene abeparvovec-xioi) or are continuing to be treated with an approved SMN2-targeted therapy (ie, nusinersen or risdiplam)
6. Body weight for age is no less than 1st percentile based on the WHO Child Growth Standards at the Screening Visit
7. Has delayed motor milestones for age attributed to SMA at the discretion of the Investigator or a CHOP-INTEND score \<55

Exclusion Criteria

1. Nutritional status that is not anticipated to be stable throughout the study or medical necessity for a gastric feeding tube, where most feeds are administered by this route
2. Major orthopedic issues such as severe scoliosis or severe contractures or interventional procedure, including spine or hip surgery, which is considered to have the potential to substantially limit the ability of the subject to be evaluated on any motor function outcome measures, within 6 months before Screening or anticipated during the study
3. Any other physical limitations (eg, the subject requires cast for contractures) that would prevent the subject from undergoing motor function outcome measures throughout the study.
Maximum Eligible Age

2 Years

Eligible Sex

ALL

Accepts Healthy Volunteers

No

Sponsors

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Scholar Rock, Inc.

INDUSTRY

Sponsor Role lead

Responsible Party

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Responsibility Role SPONSOR

Locations

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Phoenix Children's Hospital

Phoenix, Arizona, United States

Site Status RECRUITING

Stanford Neuroscience Health Center (SNHC)

Palo Alto, California, United States

Site Status RECRUITING

Children's Hospital Colorado

Aurora, Colorado, United States

Site Status RECRUITING

University of Iowa

Iowa City, Iowa, United States

Site Status RECRUITING

Atrium Health Wake Forest Baptist

Winston-Salem, North Carolina, United States

Site Status RECRUITING

University of Texas Southwestern Medical Center

Dallas, Texas, United States

Site Status RECRUITING

Neurology Rare Disease Center

Flower Mound, Texas, United States

Site Status RECRUITING

Cook Children's Medical Center

Fort Worth, Texas, United States

Site Status RECRUITING

UZ Leuven - Campus Gasthuisberg

Leuven, , Belgium

Site Status RECRUITING

CHR Citadelle

Liège, , Belgium

Site Status RECRUITING

Fondazione I.R.C.C.S. - Istituto Neurologico Carlo Besta

Milan, , Italy

Site Status RECRUITING

Centro Clinico NeMO Milano - Fondazione Serena Onlus

Milan, , Italy

Site Status RECRUITING

Hospital Sant Joan de Déu Barcelona

Barcelona, , Spain

Site Status RECRUITING

Hospital Universitari i Politecnico La Fecnic La Fe

Valencia, , Spain

Site Status RECRUITING

Countries

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United States Belgium Italy Spain

Central Contacts

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Scholar Rock

Role: CONTACT

857-259-3860

Facility Contacts

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Melony Valdez

Role: primary

602-933-2017

Wendy Ao

Role: primary

650-725-4341

Hannah Kleiner

Role: primary

720-222-1377

Carrie Stephan

Role: primary

319-356-2673

Donna Gallimore

Role: primary

336-716-3919

Holly Lawrence

Role: primary

214-456-9561

Jennifer Avelar

Role: primary

972-999-1011

Kayla Blough

Role: primary

682-303-1489

Other Identifiers

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SRK-015-005

Identifier Type: -

Identifier Source: org_study_id

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