A new mouse model of Becker muscular dystrophy

Canadian researchers have developed a new mouse model partially deleted for the gene encoding dystrophin (DMD gene) with the aim of imitating Becker muscular dystrophy:

  • a phase deletion from exons 52 to 55 was recreated in the animal using a CRISPR-Cas9 type approach (genome editing),
  • the mutant mice did not express any pathological phenomena until the 52nd week, in either skeletal or cardiac muscle,
  • beyond this period, disturbances in muscle homeostasis and exercise-induced lesions were observed,

This new mouse model appears to be close to the human pathology (BMD) and could be used to develop therapeutic protocols for this disease.

 

Generation and characterization of a novel mouse model of Becker Muscular Dystrophy with a deletion of exons 52 to 55. Perillat LOM, Wong TWY, Maino E et al. Dis Model Mech. 2024 Aug.