Becker muscular dystrophy mice showed site-specific decay of type IIa fibers with capillary change in skeletal muscle.

ABSTRACT: Becker muscular dystrophy (BMD), an X-linked muscular dystrophy, is mostly caused by an in-frame deletion of Duchenne muscular dystrophy (DMD). BMD severity varies from asymptomatic to severe, associated with the genotype of DMD. However, the underlying mechanisms remain unclear. We established BMD mice carrying three representative exon deletions: ex45-48 del., ex45-47 del., and ex45-49 del. (d45-48, d45-47, and d45-49), with high frequencies and different severities in the human BMD hotspot. All three BMD mice showed muscle weakness, muscle degeneration, and fibrosis, but these changes appeared at different times for each exon deletion, consistent with the severities obtained by the natural history study of BMD. BMD mice showed site-specific muscle changes, unlike mdx mice, which

SUBMITTER: Miyazaki D 

PROVIDER: S-EPMC11913446 | biostudies-literature | 2025 Mar

REPOSITORIES: biostudies-literature