Safety, Tolerability, Pharmacodynamic, Efficacy, and Pharmacokinetic Study of DYNE-251 in Participants With Duchenne Muscular Dystrophy Amenable to Exon 51 Skipping

NCT ID: NCT05524883

Last Updated: 2025-08-13

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

PHASE1/PHASE2

Total Enrollment

86 participants

Study Classification

INTERVENTIONAL

Study Start Date

2022-08-12

Study Completion Date

2029-11-30

Brief Summary

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The primary purpose of this study is to evaluate the safety, tolerability, and dystrophin protein levels in muscle tissue following multiple intravenous (IV) doses of DYNE-251 in participants with Duchenne muscular dystrophy (DMD) amenable to exon 51 skipping.

The study consists of 3 periods: a multiple-ascending dose (MAD) / placebo-controlled period (24 weeks), an open-label period (24 weeks) and a long-term extension (LTE) period (192 weeks).

Detailed Description

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Conditions

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Duchenne Muscular Dystrophy (DMD)

Study Design

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Allocation Method

RANDOMIZED

Intervention Model

SEQUENTIAL

Primary Study Purpose

TREATMENT

Blinding Strategy

QUADRUPLE

Participants Caregivers Investigators Outcome Assessors

Study Groups

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Placebo-Controlled MAD Period - DYNE-251

DYNE-251 will be administered once every 4 weeks (Q4W) or once every 8 weeks (Q8W) over 24 weeks.

Group Type EXPERIMENTAL

DYNE-251

Intervention Type DRUG

Administered by IV infusion

Placebo-Controlled MAD Period - Placebo

Placebo will be administered Q4W or Q8W over 24 weeks.

Group Type EXPERIMENTAL

Placebo

Intervention Type DRUG

Administered by IV infusion

Open-Label and Long-Term Extension Period - DYNE-251

DYNE-251 will be administered Q4W or Q8W for up to 192 weeks after participants complete the Placebo-Controlled MAD Period of the study.

Group Type EXPERIMENTAL

DYNE-251

Intervention Type DRUG

Administered by IV infusion

Interventions

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DYNE-251

Administered by IV infusion

Intervention Type DRUG

Placebo

Administered by IV infusion

Intervention Type DRUG

Eligibility Criteria

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Inclusion Criteria

* Age 4 to 16 years inclusive, at the time of informed consent/assent.
* Male with a confirmed diagnosis of DMD and with a mutation in the dystrophin gene characterized by exon deletion amenable to exon 51 skipping.
* Upper extremity muscle group that is amenable to muscle biopsy.
* Brooke Upper Extremity Scale score of 1 or 2.
* Ambulatory or non-ambulatory. A non-ambulatory participant must have been non-ambulatory for \<2 years before enrollment.
* Receiving a stable dosage of glucocorticoids for at least 12 weeks prior to the start of study drug administration, with the expectation of maintaining a stable dose during the Placebo-Controlled and Open-Label Periods of the study (unless dose adjustment is required by weight change).
* Left ventricular ejection fraction of ≥50% by echocardiogram or ≥55% by cardiac magnetic resonance imaging (MRI).

Exclusion Criteria

* Uncontrolled clinical symptoms and signs of congestive heart failure (CHF).
* Any change in prophylaxis/treatment for CHF within 3 months prior to the start of study treatment.
* History of major surgical procedure within 12 weeks prior to the start of study drug administration or an expectation of a major surgical procedure during the study.
* Requirement of daytime ventilator assistance.
* Percent predicted FVC \<40 % (applies only for participants who are age ≥7 years).
* Receipt of eteplirsen, or alternative exon-skipping/dystrophin-modifying therapy, within 12 weeks of randomization.
* Receipt of non-exon skipping investigational drug within 4 months before the start of study drug administration.
* Receipt of gene therapy at any time.
Minimum Eligible Age

4 Years

Maximum Eligible Age

16 Years

Eligible Sex

MALE

Accepts Healthy Volunteers

No

Sponsors

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Dyne Therapeutics

INDUSTRY

Sponsor Role lead

Responsible Party

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Responsibility Role SPONSOR

Locations

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University of California San Diego

La Jolla, California, United States

Site Status

UCLA University California of Los Angeles

Los Angeles, California, United States

Site Status

Children's Hospital Colorado

Aurora, Colorado, United States

Site Status

Rare Disease Research, LLC

Atlanta, Georgia, United States

Site Status

UMass Memorial Medical Center

Worcester, Massachusetts, United States

Site Status

Nationwide Children's Hospital

Columbus, Ohio, United States

Site Status

Shriners Hospitals for Children Portland

Portland, Oregon, United States

Site Status

Children's Hospital of Philadelphia

Philadelphia, Pennsylvania, United States

Site Status

UPMC Children's Hospital of Pittsburgh

Pittsburgh, Pennsylvania, United States

Site Status

University of Utah - PPDS

Salt Lake City, Utah, United States

Site Status

Virginia Commonwealth University

Richmond, Virginia, United States

Site Status

Children's Hospital at Westmead

Westmead, New South Wales, Australia

Site Status

Murdoch Children's Research Institute

Parkville, Victoria, Australia

Site Status

UZ Gent

Ghent, , Belgium

Site Status

UZ Leuven

Leuven, , Belgium

Site Status

CHR Citadelle

Liège, , Belgium

Site Status

London Health Sciences Centre

London, Ontario, Canada

Site Status

Children's Hospital of Eastern Ontario

Ottawa, Ontario, Canada

Site Status

CHI [Children's Health Ireland] at Temple Street Children's University Hospital

Dublin, , Ireland

Site Status

Fondazione Policlinico Universitario A Gemelli

Rome, Lazio, Italy

Site Status

Fondazione Serena Onlus - Centro Clinico NeMO

Milan, Lombardy, Italy

Site Status

Ospedale San Raffaele S.r.l. - PPDS

Milan, Lombardy, Italy

Site Status

Samsung Medical Center

Seoul, Teugbyeolsi, South Korea

Site Status

Hospital Universitario Vall d'Hebron - PPDS

Barcelona, , Spain

Site Status

Hospital Sant Joan de Déu Universidad de Barcelona

Barcelona, , Spain

Site Status

Alder Hey Children's Hospital

Liverpool, Merseyside, United Kingdom

Site Status

Royal Victoria Infirmary

Newcastle upon Tyne, Northumberland, United Kingdom

Site Status

Leeds Teaching Hospitals NHS Trust

Leeds, West Yorkshire, United Kingdom

Site Status

Bristol Childrens Hospital

Bristol, , United Kingdom

Site Status

Great Ormond Street Hospital

London, , United Kingdom

Site Status

Countries

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United States Australia Belgium Canada Ireland Italy South Korea Spain United Kingdom

Other Identifiers

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2023-510351-31-00

Identifier Type: CTIS

Identifier Source: secondary_id

ITA 362546 (model PA-000798)

Identifier Type: OTHER

Identifier Source: secondary_id

DYNE251-DMD-201

Identifier Type: -

Identifier Source: org_study_id

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