RecruitingPhase 2NCT07047144

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

A Phase 2, Double-Blind Study to Evaluate the Pharmacokinetics, Pharmacodynamics, Efficacy, and Safety of Apitegromab in Subjects <2 Years Old With Spinal Muscular Atrophy (SMA)


Sponsor

Scholar Rock, Inc.

Enrollment

52 participants

Start Date

Sep 15, 2025

Study Type

INTERVENTIONAL

Conditions

Summary

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.


Eligibility

Max Age: 2 Years

Inclusion Criteria7

  • Is \<2 years old at the time of the informed consent
  • Had a gestational age of ≥35 weeks and gestational body weight ≥2.0 kg at birth
  • Has confirmed diagnosis of 5q autosomal recessive SMA
  • Has confirmed presence of SMN2 gene copy(ies)
  • 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)
  • Body weight for age is no less than 1st percentile based on the WHO Child Growth Standards at the Screening Visit
  • Has delayed motor milestones for age attributed to SMA at the discretion of the Investigator or a CHOP-INTEND score \<55

Exclusion Criteria3

  • 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
  • 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
  • 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.

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Interventions

DRUGApitegromab

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.

DRUGNusinersen

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

DRUGRisdiplam

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


Locations(23)

Phoenix Children's Hospital

Phoenix, Arizona, United States

Children's Hospital of Orange County (CHOC)

Orange, California, United States

Stanford Neuroscience Health Center (SNHC)

Palo Alto, California, United States

Children's Hospital Colorado

Aurora, Colorado, United States

University of Iowa

Iowa City, Iowa, United States

Helen DeVos Children's Hospital at Spectrum Health

Grand Rapids, Michigan, United States

Atrium Health Wake Forest Baptist

Winston-Salem, North Carolina, United States

The Children's Hospital of Philadelphia

Philadelphia, Pennsylvania, United States

Le Bonheur Children's Hospital

Memphis, Tennessee, United States

University of Texas Southwestern Medical Center

Dallas, Texas, United States

Neurology Rare Disease Center

Flower Mound, Texas, United States

Cook Children's Medical Center

Fort Worth, Texas, United States

Texas Children's Hospital (TCH)-Clinical Care Center (CCC)

Houston, Texas, United States

UZ Gent

Ghent, East-Flanders, Belgium

UZ Leuven - Campus Gasthuisberg

Leuven, Belgium

CHR Citadelle

Liège, Belgium

Hopital Trousseau - I-Motion

Paris, France

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

Milan, Italy

Centro Clinico NeMO Milano - Fondazione Serena Onlus

Milan, Italy

Fondazione Policlinico Universitario Agostino Gemelli IRCCS

Rome, Italy

Universitair Medisch Centrum Utrecht

Utrecht, Netherlands

Hospital Sant Joan de Déu Barcelona

Barcelona, Spain

Hospital Universitari i Politecnico La Fecnic La Fe

Valencia, Spain

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NCT07047144


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