Voluntary wheel running improves molecular and functional deficits in a murine model of facioscapulohumeral muscular dystrophy.

ABSTRACT: Endurance exercise training is beneficial for skeletal muscle health, but it is unclear if this type of exercise can target or correct the molecular mechanisms of facioscapulohumeral muscular dystrophy (FSHD). Using the FLExDUX4 murine model of FSHD characterized by chronic, low levels of pathological double homeobox protein 4 (DUX4) gene expression, we show that 6 weeks of voluntary, free wheel running improves running performance, strength, mitochondrial function, and sarcolemmal repair capacity, while slowing/reversing skeletal muscle fibrosis. These improvements are associated with restored transcriptional activity of gene networks/pathways regulating actin cytoskeletal signaling, vascular remodeling, inflammation, fibrosis, and muscle mass toward wild-type (WT) levels. However,

SUBMITTER: Bittel AJ 

PROVIDER: S-EPMC10770537 | biostudies-literature | 2024 Jan

REPOSITORIES: biostudies-literature