Safety, Tolerability, Pharmacodynamic, Efficacy, and Pharmacokinetic Study of DYNE-101 in Participants With Myotonic Dystrophy Type 1

NCT ID: NCT05481879

Last Updated: 2025-11-06

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/PHASE2

Total Enrollment

116 participants

Study Classification

INTERVENTIONAL

Study Start Date

2022-09-05

Study Completion Date

2029-07-31

Brief Summary

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The primary purpose of the study is to evaluate the safety and tolerability of multiple intravenous (IV) doses of DYNE-101 administered to participants with Myotonic Dystrophy Type 1 (DM1).

The study consists of 4 periods: A Screening Period (up to 8 weeks), a Placebo-Controlled Period (24 weeks), a Treatment Period (24 weeks) and a Long-Term Extension (LTE) Period (168 weeks) in both multiple-ascending dose (MAD) and dose expansion cohorts.

Detailed Description

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Conditions

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Myotonic Dystrophy Type 1 (DM1)

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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MAD Cohort: Placebo-Controlled Period: DYNE-101

Participants will be randomized to receive ascending doses of DYNE-101, once every 4 weeks (Q4W) or once every 8 weeks (Q8W) for up to 24 weeks.

Group Type EXPERIMENTAL

DYNE-101

Intervention Type DRUG

Administered by IV infusion

MAD Cohort: Placebo-Controlled Period: Placebo

Participants will be randomized to receive DYNE-101 matching placebo, Q4W or Q8W for up to 24 weeks.

Group Type PLACEBO_COMPARATOR

Placebo

Intervention Type DRUG

Administered by IV infusion

MAD Cohort: Treatment Period: DYNE-101

Participants who receive DYNE-101 in Placebo-Controlled Period will continue to receive DYNE-101, Q4W or Q8W for up to 24 weeks.

Participants who receive placebo in Placebo-Controlled Period will receive DYNE-101, Q4W or Q8W for up to 24 weeks.

Group Type EXPERIMENTAL

DYNE-101

Intervention Type DRUG

Administered by IV infusion

Placebo

Intervention Type DRUG

Administered by IV infusion

MAD Cohort: Long-Term Extension Period: DYNE-101

Participants will receive DYNE-101, Q4W or Q8W for up to 168 weeks.

Group Type EXPERIMENTAL

DYNE-101

Intervention Type DRUG

Administered by IV infusion

Dose Expansion Cohort: Placebo-Controlled Period: DYNE-101

Participants will receive DYNE-101, Q8W for up to 24 weeks.

Group Type EXPERIMENTAL

DYNE-101

Intervention Type DRUG

Administered by IV infusion

Dose Expansion Cohort: Placebo-Controlled Period: Placebo

Participants will receive DYNE-101 matching placebo, Q8W for up to 24 weeks.

Group Type EXPERIMENTAL

Placebo

Intervention Type DRUG

Administered by IV infusion

Dose Expansion Cohort: Treatment Period: DYNE-101

Participants who receive DYNE-101 in Placebo-Controlled Period will continue to receive DYNE-101, Q8W for up to 24 weeks.

Participants who receive placebo in Placebo-Controlled Period will receive DYNE-101, Q8W for up to 24 weeks.

Group Type EXPERIMENTAL

DYNE-101

Intervention Type DRUG

Administered by IV infusion

Placebo

Intervention Type DRUG

Administered by IV infusion

Dose Expansion Cohort: Long-Term Extension Period: DYNE-101

Participants will receive DYNE-101, Q8W for up to 168 weeks.

Group Type EXPERIMENTAL

DYNE-101

Intervention Type DRUG

Administered by IV infusion

Interventions

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DYNE-101

Administered by IV infusion

Intervention Type DRUG

Placebo

Administered by IV infusion

Intervention Type DRUG

Eligibility Criteria

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Inclusion Criteria

* Diagnosis of DM1 with trinucleotide repeat size \>100.
* Age of onset of DM1 muscle symptoms ≥12 years.
* Clinically apparent myotonia equivalent to hand opening time of at least 2 seconds in the opinion of the Investigator.
* Hand grip strength and ankle dorsiflexion strength.
* Able to complete 10-MWRT, stair ascend/descend (MAD cohorts only), and 5×STS at screening without the use of assistive devices such as canes, walkers, or orthoses.

Exclusion Criteria

* History of major surgical procedure within 12 weeks prior to the start of investigative product administration or an expectation of a major surgical procedure (eg, implantation of cardiac defibrillator) during the study.
* History of anaphylaxis.
* Medical condition other than DM1 that would significantly impact ambulation or participation in functional assessments.
* Treatment with medications that can improve myotonia within a period of 5 half-lives of the medication prior to performing screening assessments.
* Electrocardiogram (ECG) with the corrected QT interval by Fridericia's Formula (QTcF) ≥450 milliseconds (ms) in men and QTcF ≥460 ms in women, PR ≥240 ms, left bundle-branch block, or a conduction defect, which is clinically significant in the opinion of the Investigator.
* Percent predicted forced vital capacity (FVC) \<50%.
* History of tibialis anterior biopsy within 3 months of Day 1 or planning to undergo tibialis anterior biopsies during study period for reasons unrelated to the study.
* Participant has a history of suicide attempt, suicidal behavior, or has any suicidal ideation within 6 months prior to Screening that meets criteria at a level of 4 or 5 of the Columbia Suicide Severity Rating Scale (C-SSRS) or who, in the opinion of the Investigator, is at significant risk to commit suicide.
* Use of glucagon-like peptide 1 (GLP-1) agonist medications including semaglutide, dulaglutide, liraglutide, exenatide, or tirzepatide within a period of 5 half-lives of the medication prior to performing screening assessments.
* Significant weight loss during study participation may impact weight-based dosing, performance on muscle function assessments, and pharmacodynamic (PD) biomarkers.
Minimum Eligible Age

18 Years

Maximum Eligible Age

65 Years

Eligible Sex

ALL

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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Indiana University School of Medicine

Indianapolis, Indiana, United States

Site Status RECRUITING

Neurology Rare Disease Center

Denton, Texas, United States

Site Status RECRUITING

St. Vincent's Hospital

Fitzroy, Victoria, Australia

Site Status RECRUITING

CHU de Nantes

Nantes, , France

Site Status RECRUITING

Institut de Myologie

Paris, , France

Site Status RECRUITING

Ludwig Maximilians University, Munich - Friedrich Baur Institut

Munich, , Germany

Site Status RECRUITING

Centro Clinico Nemo

Milan, , Italy

Site Status RECRUITING

Fondazione Policlinico Universitario A Gemelli-Rome

Rome, , Italy

Site Status RECRUITING

Radboud Medical Center

Nijmegen, , Netherlands

Site Status RECRUITING

NZCR Auckland

Auckland, , New Zealand

Site Status RECRUITING

University College London Hospitals

London, , United Kingdom

Site Status RECRUITING

John Walton Muscular Dystrophy Research Centre

Newcastle upon Tyne, , United Kingdom

Site Status RECRUITING

Salford Royal Hospital

Salford, , United Kingdom

Site Status RECRUITING

Countries

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United States Australia France Germany Italy Netherlands New Zealand United Kingdom

Central Contacts

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Dyne Clinical Trials

Role: CONTACT

Phone: +1-781-317-1919

Email: [email protected]

Facility Contacts

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Patti Hogan, BSN RN, CCRA

Role: primary

Andrea Ramirez

Role: primary

Alan Lai

Role: primary

Marie-Agnès Sourdril

Role: primary

Asma Balloumi

Role: primary

Corinna Wirner-Piotrowski

Role: primary

Maribel Evoli

Role: primary

Maria Pirozzoli

Role: primary

Eline Sanders

Role: primary

Miriam Rodrigues

Role: primary

Nikoletta Nikolenko

Role: primary

Nicola McLarty

Role: primary

Bethan Blackledge

Role: primary

Other Identifiers

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2023-510353-42-00

Identifier Type: CTIS

Identifier Source: secondary_id

DYNE101-DM1-201

Identifier Type: -

Identifier Source: org_study_id