Long-Term Safety & Efficacy of Apitegromab in Patients With SMA Who Completed Previous Trials of Apitegromab

NCT ID: NCT05626855

Last Updated: 2025-05-25

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

Get a concise snapshot of the trial, including recruitment status, study phase, enrollment targets, and key timeline milestones.

Recruitment Status

ACTIVE_NOT_RECRUITING

Clinical Phase

PHASE3

Total Enrollment

238 participants

Study Classification

INTERVENTIONAL

Study Start Date

2023-04-17

Study Completion Date

2029-05-02

Brief Summary

Review the sponsor-provided synopsis that highlights what the study is about and why it is being conducted.

The ONYX study is an Open-Label, Multicenter, Extension study that will evaluate the long-term safety and efficacy of Apitegromab in Patients with Type 2 and Type 3 SMA who have completed TOPAZ or SAPPHIRE.

Detailed Description

Dive into the extended narrative that explains the scientific background, objectives, and procedures in greater depth.

Conditions

See the medical conditions and disease areas that this research is targeting or investigating.

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

Study Design

Understand how the trial is structured, including allocation methods, masking strategies, primary purpose, and other design elements.

Allocation Method

NA

Intervention Model

SINGLE_GROUP

Open Label Extension Study
Primary Study Purpose

TREATMENT

Blinding Strategy

NONE

Study Groups

Review each arm or cohort in the study, along with the interventions and objectives associated with them.

Treatment Period

Patients who are ≥2 years of age with Type 2 and Type 3 SMA will receive apitegromab 20 mg/kg every 4 weeks by intravenous (IV) infusion during the 104-week Treatment Period

Group Type EXPERIMENTAL

Apitegromab

Intervention Type DRUG

Apitegromab (SRK-015) is an investigational, fully human immunoglobulin G4 monoclonal antibody that specifically binds to human proforms (i.e., inactive precursor forms) of myostatin, pro- and latent- myostatin, with high affinity, inhibiting activation of myostatin, a negative regulator of muscle growth and strength.

Interventions

Learn about the drugs, procedures, or behavioral strategies being tested and how they are applied within this trial.

Apitegromab

Apitegromab (SRK-015) is an investigational, fully human immunoglobulin G4 monoclonal antibody that specifically binds to human proforms (i.e., inactive precursor forms) of myostatin, pro- and latent- myostatin, with high affinity, inhibiting activation of myostatin, a negative regulator of muscle growth and strength.

Intervention Type DRUG

Eligibility Criteria

Check the participation requirements, including inclusion and exclusion rules, age limits, and whether healthy volunteers are accepted.

Inclusion Criteria

* Patients have completed the Phase 2 TOPAZ (Study SRK-015-002) trial or the Phase 3 SAPPHIRE (Study SRK-015-003) trial. (For TOPAZ, completed is defined as completion of Visit EC14 in Extension Period C or participating in TOPAZ at the time the trial is ended. For SAPPHIRE, completed is defined as completion of Visit 14 or participating in SAPPHIRE at the time the trial is ended)
* Estimated life expectancy \>2 years from the Baseline Visit (Day 1)
* Able to receive study drug infusions and provide blood samples through the use of a peripheral IV or a long-term IV access device that the patient has placed for reasons independent from the trial
* Able to adhere to the requirements of the protocol, including travel to the trial site and completing all trial procedures and trial visits
* Females of childbearing potential must have a negative pregnancy test at the Baseline Visit and agree to use at least 1 highly effective method of contraception throughout the trial and for 20 weeks after the last dose of apitegromab

Exclusion Criteria

* Patient permanently discontinued study treatment during the feeder trial (i.e., TOPAZ or SAPPHIRE)
* Nutritional status that was not stable over the past 6 months and is not anticipated to be stable throughout the trial or medical necessity for a gastric/nasogastric feeding tube, where the majority of feeds are given by this route, as assessed by the Investigator
* Patient is currently enrolled in any investigational drug trial other than TOPAZ or SAPPHIRE
* Prior history of severe hypersensitivity reaction or intolerance to SMN-targeted therapies
* Prior history of severe hypersensitivity reaction or intolerance to apitegromab
* Use of chronic daytime noninvasive ventilatory support for \>16 hours daily in the 2 weeks before dosing, or anticipated to regularly receive such daytime ventilator support chronically throughout the trial
* Any acute or comorbid condition interfering with the well-being of the patient at the patient's last visit in TOPAZ or SAPPHIRE, (including active systemic infection, the need for acute treatment, or inpatient observation due to any reason). After resolution of the condition, the patient can be enrolled in the trial if they meet all the other eligibility criteria.
* Pregnant or breastfeeding
* Any other condition or clinically significant laboratory result or ECG value that, in the opinion of the Investigator, may compromise safety or compliance, would preclude the patient from successful completion of the trial, or interfere with the interpretation of the results
Minimum Eligible Age

2 Years

Eligible Sex

ALL

Accepts Healthy Volunteers

No

Sponsors

Meet the organizations funding or collaborating on the study and learn about their roles.

Scholar Rock, Inc.

INDUSTRY

Sponsor Role lead

Responsible Party

Identify the individual or organization who holds primary responsibility for the study information submitted to regulators.

Responsibility Role SPONSOR

Locations

Explore where the study is taking place and check the recruitment status at each participating site.

Phoenix Childrens Hospital

Phoenix, Arizona, United States

Site Status

UCSD Altman Clinical and Translational Research

La Jolla, California, United States

Site Status

Children's Hospital Los Angeles

Los Angeles, California, United States

Site Status

Stanford Neuroscience Health Center

Palo Alto, California, United States

Site Status

Children's Hospital Colorado

Aurora, Colorado, United States

Site Status

Nemours Biomedical Research

Orlando, Florida, United States

Site Status

Lurie Children's Hospital of Chicago

Chicago, Illinois, United States

Site Status

University of Iowa

Iowa City, Iowa, United States

Site Status

University of Kansas Medical Center

Fairway, Kansas, United States

Site Status

Johns HopkinsHospital

Baltimore, Maryland, United States

Site Status

Boston Children's Hospital

Boston, Massachusetts, United States

Site Status

Helen DeVos Children's Hospital

Grand Rapids, Michigan, United States

Site Status

Gillette Children's Specialty Healthcare

Saint Paul, Minnesota, United States

Site Status

Washington University Medical Campus

St Louis, Missouri, United States

Site Status

Columbia University Medical Center

New York, New York, United States

Site Status

Wake Forest University School of Medicine

Winston-Salem, North Carolina, United States

Site Status

Nationwide Children's Hospital

Columbus, Ohio, United States

Site Status

Oregon Health & Science University

Portland, Oregon, United States

Site Status

The Children's Hospital of Philadelphia

Philadelphia, Pennsylvania, United States

Site Status

St. Jude Children's Research Hospital

Memphis, Tennessee, United States

Site Status

University of Texas Southwestern - Pediatric Neurology

Dallas, Texas, United States

Site Status

University of Utah

Salt Lake City, Utah, United States

Site Status

Children's Specialty Group PLLC (Children's Hospital of The King's Daughters)

Newport News, Virginia, United States

Site Status

Seattle Children's Hospital

Seattle, Washington, United States

Site Status

University of Wisconsin Hospital

Madison, Wisconsin, United States

Site Status

UZ Gent

Ghent, , Belgium

Site Status

UZ Leuven

Leuven, , Belgium

Site Status

CHR Citadelle

Liège, , Belgium

Site Status

CHRU de Lille - Hpital Jeanne de Flandre

Lille, , France

Site Status

Hopital Trousseau - I-Motion

Paris, , France

Site Status

CHU Toulouse Hopital des Enfants

Toulouse, , France

Site Status

Universitätskinderklinik Bonn, Abteilung für Neuropädiatrie und SPZ

Bonn, , Germany

Site Status

Universitatsklinikum Essen

Essen, , Germany

Site Status

Universitatsklinikum Freiburg

Freiburg im Breisgau, , Germany

Site Status

Klinikum der Universitat Munchen, Dr. von Haunersches Kinderspital, Abteilung fur Kinderneurologie und Entwicklungsneurologie

Munchen, Bayern, , Germany

Site Status

Istituto Giannina Gaslini, Centro Traslazionale di Miologia e Patologie Neurodegenerative

Genova, , Italy

Site Status

UOC NEUROLOGIA E MALATTIE NEUROMUSCOLARI A.O.U Policlinico G. Martino

Messina, , Italy

Site Status

Carlo Besta Neurological Research Institute

Milan, , Italy

Site Status

NeuroMuscular Omnicentre

Milan, , Italy

Site Status

Fondazione Policlinico Universitario A. Gemelli

Roma, , Italy

Site Status

UMC Utrecht

Utrecht, , Netherlands

Site Status

Uniwersyteckie Centrum Kliniczne

Gdansk, , Poland

Site Status

Uniwersytecki Szpital Kliniczny w Poznaniu, Oddział Kliniczny Neurologii Dzieci i Młodzieży

Poznan, , Poland

Site Status

Instytut Pomnik - Centrum Zdrowia Dziecka

Warsaw, , Poland

Site Status

Hospital Sant Joan de Deau

Barcelona, , Spain

Site Status

Hospital Universitari i Politecnic La Fe

Valencia, , Spain

Site Status

Leeds Children's Hospital Clinical Research

Leeds, , United Kingdom

Site Status

Great Ormond Street Hospital for Children

London, , United Kingdom

Site Status

University of Oxford

Oxford, , United Kingdom

Site Status

Countries

Review the countries where the study has at least one active or historical site.

United States Belgium France Germany Italy Netherlands Poland Spain United Kingdom

Other Identifiers

Review additional registry numbers or institutional identifiers associated with this trial.

SRK-015-004

Identifier Type: -

Identifier Source: org_study_id

More Related Trials

Additional clinical trials that may be relevant based on similarity analysis.

Spinraza in Adult Spinal Muscular Atrophy
NCT03709784 ACTIVE_NOT_RECRUITING