Global Open-Label Extension Study of Del-desiran for the Treatment of DM1

NCT ID: NCT07008469

Last Updated: 2025-12-22

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

ENROLLING_BY_INVITATION

Clinical Phase

PHASE3

Total Enrollment

230 participants

Study Classification

INTERVENTIONAL

Study Start Date

2025-07-25

Study Completion Date

2030-10-31

Brief Summary

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A Global Phase 3 Open-Label Extension Study to Assess the Long-Term Safety, Tolerability, and Efficacy 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 either 4-weeks or 8-weeks depending on the prior parent trial, and up to a 4-year Treatment Period. The anticipated duration is 50 months and 2 weeks (4 years and 2.5 months).

Participants will receive an intravenous infusion of del-desiran at the clinical study site every 8 weeks for a total of 7 doses per year. The final dose will occur at Year 4, Visit 7, followed by a final assessment 8 weeks after the last dose.

An additional subgroup of de novo participants will also be included in a Fixed-Dose PK cohort.

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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Myotonic Dystrophy Type 1 DM1 Myotonic Dystrophy Myotonia Myotonic Dystrophy 1 Myotonic Disorders Steinert Myotonic Dystrophy Steinert Disease

Keywords

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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 HARBOR-OLE Avidity Biosciences Avidity AOC 1001 Del-desiran Delpacibart etedesiran HARBOR

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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Del-desiran

Del-desiran (AOC 1001) will be administered seven times per year for up to 4 years.

Group Type EXPERIMENTAL

Del-desiran (AOC 1001)

Intervention Type DRUG

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

Interventions

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Del-desiran (AOC 1001)

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

Intervention Type DRUG

Eligibility Criteria

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Inclusion Criteria

* Completion of any prior AOC 1001 studies with satisfactory completion of dosing and follow-up assessments and satisfactory compliance with the protocol requirements of the parent study, as determined by the Investigator.


* Clinical and genetic diagnosis of DM1 and clinically significant hand myotonia.

Exclusion Criteria

* Breastfeeding, pregnancy, or intent to become pregnant during the study.
* Unwilling to comply with contraceptive requirements.
* Any new conditions or worsening of existing condition that in the opinion of the Investigator would make the participant unsuitable for the study.

FIXED-DOSE PK COHORT


* Breastfeeding, pregnancy, or intent to become pregnant during the study
* Unwilling 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
Minimum Eligible Age

16 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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University of California, Los Angeles (UCLA)

Los Angeles, California, United States

Site Status

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

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 Rochester Medical Center

Rochester, New York, 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

Aomori Hospital

Aomori, Aomori, Japan

Site Status

Countries

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United States Japan

Other Identifiers

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AOC 1001-CS4

Identifier Type: -

Identifier Source: org_study_id