Global Study of Del-desiran for the Treatment of DM1

NCT ID: NCT06411288

Last Updated: 2026-01-22

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

159 participants

Study Classification

INTERVENTIONAL

Study Start Date

2024-05-30

Study Completion Date

2026-09-30

Brief Summary

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A Phase 3 Randomized, Double-Blind, Placebo-Controlled, Global Study to Evaluate the Efficacy and Safety of Intravenous Delpacibart Etedesiran (abbreviated del-desiran, formerly AOC 1001) for the Treatment of Myotonic Dystrophy Type 1

Detailed Description

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The study consists of a Screening Period of up to 6 weeks and 54-week Treatment Period. The anticipated duration is approximately 60 weeks.

Participants will be randomized to receive an intravenous infusion of either del-desiran or placebo at the clinical study site every 8 weeks for a total of 7 doses. The final dose will occur at Week 48, followed by a final assessment at Week 54.

After completion of Week 54 assessments, eligible participants will have the option to enroll into an open label extension (OLE) study, pending regulatory approval.

An Independent Data Monitoring Committee (IDMC) comprised of members independent and external to the Sponsor will review safety, tolerability, and efficacy (as needed) data of this study at regular intervals.

Conditions

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DM1 Myotonic Dystrophy Myotonic Dystrophy 1 Myotonia Myotonic Dystrophy Type 1 (DM1) Dystrophy Myotonic Myotonic Disorders Steinert Disease Steinert Myotonic 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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Del-desiran

Del-desiran (AOC 1001) will be administered seven times

Group Type EXPERIMENTAL

AOC 1001 (del-desiran)

Intervention Type DRUG

Del-desiran will be administered by intravenous (IV) infusion.

Placebo

Saline will be administered seven times

Group Type PLACEBO_COMPARATOR

Placebo

Intervention Type DRUG

Placebo will be administered by intravenous (IV) infusion.

Interventions

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AOC 1001 (del-desiran)

Del-desiran will be administered by intravenous (IV) infusion.

Intervention Type DRUG

Placebo

Placebo will be administered by intravenous (IV) infusion.

Intervention Type DRUG

Eligibility Criteria

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Inclusion Criteria

* Clinical and genetic diagnosis (CTG repeat ≥ 100) of DM1
* Ability to walk independently (orthoses and ankle braces allowed) for at least 10 meters at screening

Exclusion Criteria

* Breastfeeding, pregnancy, or intent to become pregnant during the study
* Unwilling or unable to comply with contraceptive requirements
* Abnormal lab values, conditions or diseases that would make the participant unsuitable for the study
* Diabetes that is not adequately controlled
* History of decompensated heart failure within 3 months of screening. Participants with preexisting pacemaker/ICD are not excluded.
* Body Mass Index \> 35 kg/m2 at Screening
* Recently treated with an investigational drug or biological agent
* Treatment with anti-myotonic medication within 5 half-lives or 14 days of baseline, whichever is longer, prior to baseline.
Minimum Eligible Age

16 Years

Maximum Eligible Age

65 Years

Eligible Sex

ALL

Accepts Healthy Volunteers

No

Sponsors

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Avidity Biosciences, Inc.

INDUSTRY

Sponsor Role lead

Responsible Party

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Responsibility Role SPONSOR

Locations

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Stanford University

Stanford, California, United States

Site Status

University of Colorado

Denver, Colorado, United States

Site Status

University of Florida

Gainesville, Florida, United States

Site Status

University Research Center of South Florida

Tampa, Florida, United States

Site Status

Indiana University (IU)

Indianapolis, Indiana, United States

Site Status

Kansas University Medical Center

Kansas City, Kansas, United States

Site Status

Kennedy Krieger Institute

Baltimore, Maryland, United States

Site Status

Beth Israel Deaconess Medical Center

Boston, Massachusetts, United States

Site Status

University of Minnesota

Minneapolis, Minnesota, United States

Site Status

University of Rochester Medical Center

Rochester, New York, United States

Site Status

Duke University Medical Center

Durham, North Carolina, United States

Site Status

Wake Forest

Winston-Salem, North Carolina, United States

Site Status

University of Cincinnati Gardner Neuroscience Institute

Cincinnati, Ohio, United States

Site Status

Ohio State University

Columbus, Ohio, United States

Site Status

University of Pennsylvania

Philadelphia, Pennsylvania, United States

Site Status

Houston Methodist Neurological Institute

Houston, Texas, United States

Site Status

Virginia Commonwealth University

Richmond, Virginia, United States

Site Status

University of Washington

Seattle, Washington, United States

Site Status

The Ottawa Hospital

Ottawa, Ontario, Canada

Site Status

Montreal Neurological Institute

Montreal, Quebec, Canada

Site Status

Aarhus University Hospital

Aarhus N, , Denmark

Site Status

Rigshospitalet

Copenhagen, , Denmark

Site Status

AP-HP Hopital Pitie-Salpetriere

Paris, , France

Site Status

Klinikum der Ludwig-Maximilians-Universitaet Muenchen

Munich, , Germany

Site Status

Fondazione Serena Onlus - Centro Clinico NeMO Milano

Milan, , Italy

Site Status

Aomori Hospital

Aomori, Aomori, Japan

Site Status

National Hospital Organization Osaka Toneyama Medical Center

Osaka, , Japan

Site Status

Osaka University Hospital

Osaka, , Japan

Site Status

National Center of Neurology and Psychiatry

Tokyo, , Japan

Site Status

Maastricht University Medical Center

Maastricht, , Netherlands

Site Status

Stichting Radboud Universitair Medisch Centrum

Nijmegen, , Netherlands

Site Status

Hospital Universitario Donostia

Donostia / San Sebastian, , Spain

Site Status

University College London Hospital

London, , United Kingdom

Site Status

St. Georges University Hospitals NHS Foundation Trust

London, , United Kingdom

Site Status

Countries

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United States Canada Denmark France Germany Italy Japan Netherlands Spain United Kingdom

Other Identifiers

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AOC 1001-CS3

Identifier Type: -

Identifier Source: org_study_id

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