Advancing Efficacy, Longevity, and Safety of Gene Editing Therapies for DMD

Abstract

Adeno-associated viral (AAV) vector-based gene therapies are generating encouraging results in Duchenne muscular dystrophy (DMD) trials. However, these approaches do not fully restore muscle function and therapeutic benefits may be lost over time due to muscle turnover following maintenance, or trauma. Gene editing (CRISPR) offers a promising alternative to correct dystrophin production from the patient’s own gene. Here, long-term therapeutic outcome could benefit from repairing mutations in both existing muscles and in muscle stem cells (SCs) to ensure that dystrophin continues to be produced in case of muscle injury and regeneration. While early studies have shown that muscle and SCs can be targeted with AAV-CRISPR, efficient and safe approaches for more straightforward clinical translation have yet to be demonstrated. Here we propose developing methods to permanently restore dystrophin expression in striated muscle by correcting DMD-causing mutations in both existing muscle and in SCs. We will also develop sensitive switches for turning gene editing On and Off, with the purpose of reduced risks of unintended editing or development of immune responses within treated patient muscles. Overall, these studies are designed to achieve significant long-term therapeutic improvements of DMD symptoms.

Document Details

Document Type
DoD Grant Award
Publication Date
Jan 04, 2024
Source ID
HT94252310575

Entities

People

  • Niclas Bengtsson

Organizations

  • United States Army
  • University of Washington

Tags

Fields of Study

  • Biology
  • Medicine

Readers

  • Immunology and Pathology
  • Molecular and Cellular Biology
  • Oncology

Technology Areas

  • Biotechnology