A Study to Evaluate the Efficacy and Safety of Taldefgrobep Alfa in Participants With Spinal Muscular Atrophy

NCT ID: NCT05337553

Last Updated: 2025-08-26

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

ACTIVE_NOT_RECRUITING

Clinical Phase

PHASE3

Total Enrollment

269 participants

Study Classification

INTERVENTIONAL

Study Start Date

2022-07-06

Study Completion Date

2026-01-31

Brief Summary

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This trial will study the efficacy and safety of taldefgrobep alfa as an adjunctive therapy for participants who are already taking a stable dose of nusinersen and/or risdiplam and/or have a history of onasemnogene abeparvovec, compared to placebo.

Detailed Description

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Myostatin is a negative regulator of muscle growth. Blocking myostatin activity has been shown to increase muscle size and function. Taldefgrobep alfa directly blocks myostatin activity and was well tolerated in other clinical studies. In combination with medications that increase the amount of SMN protein in the body, taldefgrobep alfa has the potential to further improve motor function and clinical measures for people living with SMA.

Conditions

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Spinal Muscular Atrophy Neuromuscular Diseases SMA

Study Design

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Allocation Method

RANDOMIZED

Intervention Model

PARALLEL

Primary Study Purpose

TREATMENT

Blinding Strategy

QUADRUPLE

Participants Caregivers Investigators Outcome Assessors

Study Groups

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taldefgrobep alfa

taldefgrobep alfa - Double-blind (DB) Phase: Participants receive weight based 35 mg/50 mg weekly subcutaneous injection for 48-week DB phase.

taldefgrobep alfa/taldefgrobep alfa - Extension Phase: Participants receive weight based 35 mg/50 mg weekly subcutaneous injection for 48-week Open label Extension (OLE) phase.

Group Type EXPERIMENTAL

taldefgrobep alfa

Intervention Type DRUG

DB Phase: 35 mg/50 mg weekly subcutaneous injection

taldefgrobep alfa

Intervention Type DRUG

Extension Phase: 35 mg/50 mg weekly subcutaneous injection

Placebo

Placebo - Double-blind (DB) Phase: Participants receive weight based 35 mg/50 mg weekly subcutaneous injection for 48-week DB phase.

Placebo/taldefgrobep alfa - Extension Phase: Participants who receive placebo during DB phase, receive weight based 35 mg/50 mg weekly subcutaneous taldefgrobep alfa injection for 48-week OLE phase.

Group Type PLACEBO_COMPARATOR

Placebo

Intervention Type DRUG

DB Phase: matching placebo 35 mg/50 mg weekly subcutaneous injection

taldefgrobep alfa

Intervention Type DRUG

Extension Phase: 35 mg/50 mg weekly subcutaneous injection

Interventions

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taldefgrobep alfa

DB Phase: 35 mg/50 mg weekly subcutaneous injection

Intervention Type DRUG

Placebo

DB Phase: matching placebo 35 mg/50 mg weekly subcutaneous injection

Intervention Type DRUG

taldefgrobep alfa

Extension Phase: 35 mg/50 mg weekly subcutaneous injection

Intervention Type DRUG

Other Intervention Names

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BHV-2000 BMS-986089 BHV-2000 BMS-986089

Eligibility Criteria

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Inclusion Criteria

* Spinal Muscular Atrophy confirmed by genetic diagnosis of 5q-autosomal recessive SMA as well as SMN2 copy number
* Ambulant or Non-Ambulant
* Treated with an SMA disease-modifying therapy and anticipated to remain on that same treatment regimen and dose throughout the trial including nusinersen and/or risdiplam and/or a history of onasemnogene abeparvovec

Exclusion Criteria

* Cannot have previously taken anti-myostatin therapies
* Must weigh at least 15kg
* Respiratory insufficiency, defined by the medical necessity for invasive or non-invasive ventilation for daytime treatment while awake (use overnight or during daytime naps is acceptable)
* History of Spinal Fusion within 6 months of Screening. MAGEC rod nonsurgical adjustments are allowed during the study
* Presence of an implanted shunt for the drainage of CSF or an implanted central nervous system (CNS) catheter
Minimum Eligible Age

4 Years

Maximum Eligible Age

21 Years

Eligible Sex

ALL

Accepts Healthy Volunteers

No

Sponsors

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Biohaven Pharmaceuticals, Inc.

INDUSTRY

Sponsor Role lead

Responsible Party

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Responsibility Role SPONSOR

Principal Investigators

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Lindsey Lair, MD

Role: STUDY_DIRECTOR

Biohaven Pharmaceuticals, Inc.

Locations

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Phoenix Children's

Phoenix, Arizona, United States

Site Status

UCSD & Rady Children's

La Jolla, California, United States

Site Status

Children's Hospital of Los Angeles

Los Angeles, California, United States

Site Status

UCSF Benioff Children's Hospital, Medical Center

San Francisco, California, United States

Site Status

Children's Hospital Colorado

Aurora, Colorado, United States

Site Status

UF Health, Shands Hospital

Gainesville, Florida, United States

Site Status

Rare Disease Research

Atlanta, Georgia, United States

Site Status

Northwestern University - Feinberg School of Medicine - Ann & Robert H. Lurie Children's Hospital of Chicago

Chicago, Illinois, United States

Site Status

Indiana University -Riley Research

Indianapolis, Indiana, 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

Boston Children's Hospital - Harvard

Boston, Massachusetts, United States

Site Status

BSHS Office of Research

Grand Rapids, Michigan, United States

Site Status

Washington University in St. Louis

St Louis, Missouri, United States

Site Status

Columbia University Medical Center

New York, New York, United States

Site Status

Stony Brook University Hospital

Stony Brook, New York, United States

Site Status

Duke University Medicine

Durham, North Carolina, United States

Site Status

Cincinnati Children's Hospital Medical Center

Cincinnati, Ohio, United States

Site Status

Nationwide Children's Hospital

Columbus, Ohio, United States

Site Status

Penn State College of Medicine

Hershey, Pennsylvania, United States

Site Status

CHOP Children's Hospital of Philadelphia

Philadelphia, Pennsylvania, United States

Site Status

UPMC Children's Hospital of Pittsburgh

Pittsburgh, Pennsylvania, United States

Site Status

Vanderbilt University Medical Center

Nashville, Tennessee, United States

Site Status

UT Pediatric Neurosciences/Dell Children's Medical Center

Austin, Texas, United States

Site Status

Neurology Rare Disease Center

Denton, Texas, United States

Site Status

Cook Children's Hospital

Fort Worth, Texas, United States

Site Status

University of Virginia Children's Hospital

Charlottesville, Virginia, United States

Site Status

Children's Hospital of The King's Daughters

Norfolk, Virginia, United States

Site Status

MultiCare Institute of Research and Innovation

Tacoma, Washington, United States

Site Status

Medical College of Wisconsin

Milwaukee, Wisconsin, United States

Site Status

University Hospital Antwerp

Edegem, , Belgium

Site Status

University Hospital Ghent

Ghent, , Belgium

Site Status

University Hospital Leuven

Leuven, , Belgium

Site Status

University Hospital Brno - Dept. of Pediatric Neurology

Brno, , Czechia

Site Status

Motol University Hospital

Prague, , Czechia

Site Status

University Hospital Essen (Public-Law Institution) - Dept. of Pediatrics I

Essen, , Germany

Site Status

University Hospital Freiburg, Center For Children and Adolescent Medicine, Dept. of Neuropediatrics and Muscle Disorders

Freiburg im Breisgau, , Germany

Site Status

Dr. Von Haunersches Children'S Hospital - Lmu Munich

Munich, , Germany

Site Status

Irccs Institute of Neurological Sciences of Bologna - Bellaria Hospital

Bologna, , Italy

Site Status

Nemo-Brescia Clinical Center For Neuromuscular Diseases

Gussago, , Italy

Site Status

IRCCS NEUROLOGICAL INSTITUTE C. MONDINO CHILD and NEUROPSYCHIATRIC UNIT

Pavia, , Italy

Site Status

Bambino Gesù Children'S Research Hospital Irccs - San Paolo Office Dept. of Neuroscience

Roma, , Italy

Site Status

University Medical Center Utrecht

Utrecht, , Netherlands

Site Status

University Clinical Centre in Gdansk - Dept. of Developmental Neurology

Gdansk, , Poland

Site Status

Heliodor Swiecicki Clinical Hospital At Medical University - Child and Adolescents Neurology Clinic

Poznan, , Poland

Site Status

The Children'S Memorial Health Institute - Dept. of Neurology and Epileptology

Warsaw, , Poland

Site Status

T. Marciniak Lower Silesian Specialist Hospital, Pediatric Neurology Dept.

Wroclaw, , Poland

Site Status

Donostia University Hospital

Donostia / San Sebastian, , Spain

Site Status

Hospital Sant Joan de Déu

Esplugues de Llobregat, , Spain

Site Status

Maternal-Child'S Hospital of Málaga, Regional University Hospital - Pediatric Neurology Unit

Málaga, , Spain

Site Status

La Fe University and Polytechnic Hospital

Valencia, , Spain

Site Status

Royal Hospital For Children

Glasgow, Scotland, United Kingdom

Site Status

John Radcliffe Hospital

Oxford, , United Kingdom

Site Status

Countries

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United States Belgium Czechia Germany Italy Netherlands Poland Spain United Kingdom

Other Identifiers

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BHV2000-301

Identifier Type: -

Identifier Source: org_study_id

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