A Gene Transfer Therapy Study to Evaluate the Safety and Efficacy of Delandistrogene Moxeparvovec (SRP-9001) in Participants With Duchenne Muscular Dystrophy (DMD)

NCT ID: NCT05096221

Last Updated: 2025-07-08

Study Results

Results available

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Basic Information

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Recruitment Status

COMPLETED

Clinical Phase

PHASE3

Total Enrollment

126 participants

Study Classification

INTERVENTIONAL

Study Start Date

2021-10-27

Study Completion Date

2024-10-25

Brief Summary

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The study will evaluate the safety and efficacy of gene transfer therapy in boys with DMD. It is a randomized, double-blind, placebo-controlled study. The participants who are randomized to the placebo arm will have an opportunity for treatment with gene transfer therapy at the beginning of the second year.

Detailed Description

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Conditions

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Duchenne Muscular Dystrophy

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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Delandistrogene Moxeparvovec followed by Placebo

Participants will receive single intravenous (IV) infusion of delandistrogene moxeparvovec on Day 1. Then, participants will receive a single IV infusion of matching placebo at Year 2.

Group Type EXPERIMENTAL

delandistrogene moxeparvovec

Intervention Type GENETIC

Single IV infusion of delandistrogene moxeparvovec.

placebo

Intervention Type GENETIC

Single IV infusion of matching placebo.

Placebo followed by Delandistrogene Moxeparvovec

Participants will receive matching placebo IV infusion on Day 1. Then, participants will have the opportunity to receive a single IV infusion of delandistrogene moxeparvovec at Year 2.

Group Type PLACEBO_COMPARATOR

delandistrogene moxeparvovec

Intervention Type GENETIC

Single IV infusion of delandistrogene moxeparvovec.

placebo

Intervention Type GENETIC

Single IV infusion of matching placebo.

Interventions

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delandistrogene moxeparvovec

Single IV infusion of delandistrogene moxeparvovec.

Intervention Type GENETIC

placebo

Single IV infusion of matching placebo.

Intervention Type GENETIC

Other Intervention Names

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SRP-9001 delandistrogene moxeparvovec-rokl ELEVIDYS

Eligibility Criteria

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Inclusion Criteria

* Is ambulatory and from 4 to under 8 years of age at time of randomization.
* Definitive diagnosis of DMD based on documented clinical findings and prior genetic testing.
* Ability to cooperate with motor assessment testing.
* Stable daily dose of oral corticosteroids for at least 12 weeks prior to Screening, and the dose is expected to remain constant throughout the study (except for modifications to accommodate changes in weight).
* rAAVrh74 antibody titers are not elevated as per protocol-specified requirements.
* A pathogenic frameshift mutation or premature stop codon contained between exons 18 and 79 (inclusive), with the exception of mutation fully contained within exon 45.

Exclusion Criteria

* Exposure to gene therapy, investigational medication, or any treatment designed to increase dystrophin expression within protocol specified time limits.
* Abnormality in protocol-specified diagnostic evaluations or laboratory tests.
* Presence of any other clinically significant illness, medical condition, or requirement for chronic drug treatment that in the opinion of the Investigator creates unnecessary risk for gene transfer.
Minimum Eligible Age

4 Years

Maximum Eligible Age

7 Years

Eligible Sex

MALE

Accepts Healthy Volunteers

No

Sponsors

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Hoffmann-La Roche

INDUSTRY

Sponsor Role collaborator

Sarepta Therapeutics, Inc.

INDUSTRY

Sponsor Role lead

Responsible Party

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Responsibility Role SPONSOR

Principal Investigators

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Medical Director

Role: STUDY_DIRECTOR

Sarepta Therapeutics, Inc.

Locations

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Arkansas Children's

Little Rock, Arkansas, United States

Site Status

UC San Diego Altman Clinical and Translational Research Institute

La Jolla, California, United States

Site Status

UCLA Medical Center

Los Angeles, California, United States

Site Status

Lucile Packard Children's Hospital at Stanford

Palo Alto, California, United States

Site Status

University of California, Davis

Sacramento, California, United States

Site Status

Children's Hospital Colorado

Aurora, Colorado, United States

Site Status

University of Florida

Gainesville, Florida, United States

Site Status

Lurie Children's Hospital of Chicago

Chicago, Illinois, United States

Site Status

University of Iowa Stead Family Children's Hospital

Iowa City, Iowa, United States

Site Status

The Johns Hopkins Hospital

Baltimore, Maryland, United States

Site Status

Boston Children's Hospital

Boston, Massachusetts, United States

Site Status

Washington University of St. Louis

St Louis, Missouri, United States

Site Status

Columbia University/NYPH

New York, New York, United States

Site Status

University of Rochester

Rochester, New York, United States

Site Status

Duke University Medical Center, Lenox Baker Children's Hospital

Durham, 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

Vanderbilt University Medical Center

Nashville, Tennessee, United States

Site Status

University of Texas Southwestern

Dallas, Texas, United States

Site Status

University of Utah Hospital

Salt Lake City, Utah, United States

Site Status

Children's Hospital of the King's Daughters

Norfolk, Virginia, United States

Site Status

Children's Wisconsin

Milwaukee, Wisconsin, United States

Site Status

University Hospital Ghent

Ghent, , Belgium

Site Status

LMU - Klinikum der Universitaet Muenchen - Kinderklinik und

Bayern, , Germany

Site Status

Universitätsklinikum Essen - Klinik für Kinderheilkunde I

Essen, , Germany

Site Status

University Hospital Hamburg- Eppendorf

Hamburg, , Germany

Site Status

Hong Kong Children's Hospital

Kowloon, , Hong Kong

Site Status

IRCCS Istituto G.Gaslini, U.O.

Genoa, , Italy

Site Status

UOC Neurologia, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico

Milan, , Italy

Site Status

UOC Neuropsichiatria Infantile, Area Salute del Bambino, Fondazione Policlinico Universitario A. Gamelli IRCCS

Roma, , Italy

Site Status

Kobe University Hospital

Kobe, , Japan

Site Status

National Center for Child Health and Development

Tokyo, , Japan

Site Status

Tokyo Women's Medical University Hospital - Pediatrics

Tokyo, , Japan

Site Status

National Center of Neurology and Psychiatry

Tokyo, , Japan

Site Status

Hospital Universitari i Politécnico La Fe

Valencia, Comunidad Valencia, Spain

Site Status

Hospital Sant Joan de Déu

Barcelona, , Spain

Site Status

Kaohsiung Medical University Chung-Ho Memorial Hospital

Kaohsiung City, , Taiwan

Site Status

National Taiwan University Hospital

Taipei, , Taiwan

Site Status

Oxford University Hospitals NHS Foundation Trust

Headington, Oxford, United Kingdom

Site Status

Great Ormond Street Hospital for Children NHS Foundation Trust

London, , United Kingdom

Site Status

The Newcastle Upon Tyne NHS Hospital NHS Foundation Trust, Royal Victoria Infirmary

Newcastle upon Tyne, , United Kingdom

Site Status

Countries

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United States Belgium Germany Hong Kong Italy Japan Spain Taiwan United Kingdom

References

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Mendell JR, Muntoni F, McDonald CM, Mercuri EM, Ciafaloni E, Komaki H, Leon-Astudillo C, Nascimento A, Proud C, Schara-Schmidt U, Veerapandiyan A, Zaidman CM, Guridi M, Murphy AP, Reid C, Wandel C, Asher DR, Darton E, Mason S, Potter RA, Singh T, Zhang W, Fontoura P, Elkins JS, Rodino-Klapac LR. AAV gene therapy for Duchenne muscular dystrophy: the EMBARK phase 3 randomized trial. Nat Med. 2025 Jan;31(1):332-341. doi: 10.1038/s41591-024-03304-z. Epub 2024 Oct 9.

Reference Type RESULT
PMID: 39385046 (View on PubMed)

Vandenborne K, Walter GA, Straub V, Willcocks RJ, Forbes SC, Mercuri EM, Muntoni F, Ding K, Ennamuri S, Reid C, Murphy AP, Manfrini M, Mendell JR, Elkins JS, Rodino-Klapac LR. Quantitative Muscle Magnetic Resonance Outcomes in Patients With Duchenne Muscular Dystrophy: An Exploratory Analysis From the EMBARK Randomized Clinical Trial. JAMA Neurol. 2025 Jul 1;82(7):734-744. doi: 10.1001/jamaneurol.2025.0992.

Reference Type DERIVED
PMID: 40354061 (View on PubMed)

McDonald CM, Elkins JS, Dharmarajan S, Gooch K, Ciobanu T, Lansdall CJ, Murphy AP, McDougall F, Mercuri EM, Audhya I; EMBARK Study Group. Caregiver Global Impression Observations from EMBARK: A Phase 3 Study Evaluating Delandistrogene Moxeparvovec in Ambulatory Patients with Duchenne Muscular Dystrophy. Neurol Ther. 2025 Feb;14(1):211-225. doi: 10.1007/s40120-024-00685-8. Epub 2024 Nov 26.

Reference Type DERIVED
PMID: 39589719 (View on PubMed)

Provided Documents

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Document Type: Study Protocol and Statistical Analysis Plan

View Document

Related Links

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Other Identifiers

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2019-003374-91

Identifier Type: EUDRACT_NUMBER

Identifier Source: secondary_id

SRP-9001-301

Identifier Type: -

Identifier Source: org_study_id

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