RecruitingPhase 1Phase 2NCT06907875

A First-in-human Study of EPI-321 in Facioscapulohumeral Muscular Dystrophy

A Phase 1/2 Open-label Dose-escalation Study to Evaluate the Safety, Tolerability, and Biological Activity of EPI-321, an AAVrh74-delivered Epigenetic Editing Therapy in Adult FSHD Patients


Sponsor

Epicrispr Biotechnologies, Inc.

Enrollment

12 participants

Start Date

May 8, 2025

Study Type

INTERVENTIONAL

Conditions

Summary

The goal of this clinical trial is to learn how safe and tolerable EPI-321 is and whether there may be early signs it is working in male or female adult (18 to 75 years) participants with facioscapulohumeral muscular dystrophy (FSHD) Type 1 condition. The main questions it aims to answer are: How safe is EPI-321 and how well can people handle it over time? How does EPI-321 interact with its target and does it show early signs of working? Participants will receive a single dose of EPI-321 through a vein while being closely watched in a hospital and visit the clinic regularly for tests and checkups for about 5 years after getting EPI-321.


Eligibility

Min Age: 18 YearsMax Age: 75 Years

Inclusion Criteria6

  • Able and willing to provide informed consent
  • Male or female 18 to 75 years of age
  • Clinical diagnosis of FSHD with genetic Type 1
  • FSHD Ricci clinical severity score 2 to 4 (on 5-point scale)
  • Has adequate liver function
  • Has adequate kidney function

Exclusion Criteria5

  • Has an anti-AAVrh74 total binding antibody titer > 1:400
  • Requires a walker or wheelchair for ambulation
  • Pregnant and/or breastfeeding at baseline or is planning to become pregnant during the first 12 months following EPI-321 administration
  • Has FSHD Type 2
  • Has a concurrent or past medical conditions could jeopardize the safety of the participant

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Interventions

BIOLOGICALEPI-321

EPI-321 IV Infusion


Locations(7)

David Geffen School of Medicine at University of California, Los Angeles

Los Angeles, California, United States

Rare Disease Research

Atlanta, Georgia, United States

Kennedy Krieger Institute, Center for Genetic Muscle Disorders

Baltimore, Maryland, United States

University of Massachusetts Chan Medical School

Worcester, Massachusetts, United States

Utah Program for Inherited Neuromuscular Disorders - University of Utah

Salt Lake City, Utah, United States

Royal Alfred Hospital

Sydney, New South Wales, Australia

Pacific Clinical Research Network

Auckland, New Zealand

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NCT06907875


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