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

NCT ID: NCT04626674

Last Updated: 2025-12-29

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

PHASE1

Total Enrollment

83 participants

Study Classification

INTERVENTIONAL

Study Start Date

2020-11-23

Study Completion Date

2028-02-29

Brief Summary

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This is an open-label gene transfer therapy study evaluating the safety of and expression from delandistrogene moxeparvovec in participants with DMD. The maximum participant duration for this study is 156 weeks.

Detailed Description

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Conditions

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Muscular Dystrophy, Duchenne

Keywords

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Duchenne Muscular Dystrophy Gene-Delivery DMD Ambulatory Non-ambulatory Pediatric Dystrophin

Study Design

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Allocation Method

NA

Intervention Model

SINGLE_GROUP

Primary Study Purpose

TREATMENT

Blinding Strategy

NONE

Study Groups

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Delandistrogene Moxeparvovec

Participants will receive a single intravenous (IV) infusion of delandistrogene moxeparvovec on Day 1.

Group Type EXPERIMENTAL

delandistrogene moxeparvovec

Intervention Type GENETIC

Single IV infusion of delandistrogene moxeparvovec

Interventions

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delandistrogene moxeparvovec

Single IV infusion of delandistrogene moxeparvovec

Intervention Type GENETIC

Other Intervention Names

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SRP-9001 delandistrogene moxeparvovec-rokl ELEVIDYS

Eligibility Criteria

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Inclusion Criteria

* For Cohorts 1-8: Has a definitive diagnosis of DMD based on documented clinical findings and prior genetic testing.
* Cohort 1: Is ambulatory, and ≥4 to \<8 years of age at the time of Screening.
* Cohort 2: Is ambulatory, and ≥8 to \<18 years of age at the time of Screening.
* Cohort 3: Non-ambulatory per protocol specified criteria at the time of Screening.
* Cohort 4: Is ambulatory and ≥3 to \<4 years of age at the time of Screening.
* Cohort 5a: Is ambulatory and ≥4 to \<9 years of age with time to rise from the floor ≤7 seconds at the screening visit.
* Cohort 5b: Non-ambulatory per protocol specified criteria at the time of Screening.
* Cohort 6: Is ambulatory, and ≥2 to \<3 years of age at the time of Screening.
* Cohort 7: Non-ambulatory per protocol-specified criteria at the time of Screening.
* Cohort 8: Non-ambulatory per protocol-specified criteria at the time of Screening, has a performance upper limb (PUL) entry item score ≥3 at the Screening visit and has a total PUL score of ≥20 and ≤40 at the time of Screening.
* Ability to cooperate with motor assessment testing.
* Cohorts 1, 2, 3, 5, 7 and 8 only: Stable dose equivalent of oral glucocorticoids for at least 12 weeks before screening and the dose is expected to remain constant (except for modifications to accommodate changes in weight) throughout the first year of the study.
* Cohorts 4 and 6: Do not yet require use of chronic steroids for treatment of their DMD, in the opinion of the Investigator, and are not receiving steroids at the time of Screening.
* rAAVrh74 antibody titers are not elevated as per protocol-specified requirements.

Exclusion Criteria

* Has a concomitant illness, autoimmune disease, chronic drug treatment, and/or cognitive delay/impairment that in the opinion of the Investigator creates unnecessary risks for gene transfer.
* 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.
* Cohort 8: Any confounding factors that would prevent the use of oral sirolimus including a known hypersensitivity to sirolimus or any of its excipients.
Minimum Eligible Age

2 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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Stanford University

Palo Alto, California, United States

Site Status RECRUITING

University of California, Davis

Sacramento, California, United States

Site Status RECRUITING

Washington University in St. Louis

St Louis, Missouri, United States

Site Status RECRUITING

Nationwide Children's Hospital

Columbus, Ohio, United States

Site Status RECRUITING

Children's Hospital of The King's Daughters

Norfolk, Virginia, United States

Site Status RECRUITING

Countries

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United States

Central Contacts

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Sarepta Therapeutics Inc., For Clinical Trial Information, Select Option 4

Role: CONTACT

Phone: 1-888-SAREPTA (1-888-727-3782)

Email: [email protected]

Facility Contacts

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Role: primary

Role: primary

Role: primary

References

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Potter RA, Moeller IH, Khan S, Haegel H, Hollenstein A, Steiner G, Wandel C, Murphy AP, Asher DR, Palatinsky E, Griffin DA, Mason S, Iannaccone ST, Zaidman CM, Rodino-Klapac LR. Immunologic investigations into transgene directed immune-mediated myositis following delandistrogene moxeparvovec gene therapy. Sci Rep. 2025 Jan 2;15(1):4. doi: 10.1038/s41598-024-84077-w.

Reference Type DERIVED
PMID: 39747998 (View on PubMed)

Zaidman CM, Proud CM, McDonald CM, Lehman KJ, Goedeker NL, Mason S, Murphy AP, Guridi M, Wang S, Reid C, Darton E, Wandel C, Lewis S, Malhotra J, Griffin DA, Potter RA, Rodino-Klapac LR, Mendell JR. Delandistrogene Moxeparvovec Gene Therapy in Ambulatory Patients (Aged >/=4 to <8 Years) with Duchenne Muscular Dystrophy: 1-Year Interim Results from Study SRP-9001-103 (ENDEAVOR). Ann Neurol. 2023 Nov;94(5):955-968. doi: 10.1002/ana.26755. Epub 2023 Sep 7.

Reference Type DERIVED
PMID: 37539981 (View on PubMed)

Other Identifiers

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SRP-9001-103

Identifier Type: -

Identifier Source: org_study_id