Mitochondrial dysfunction and consequences in calpain-3-deficient muscle.
Vanessa E JahnkeJennifer M PetersonJack H Van Der MeulenJessica BoehlerKitipong UaesoontrachoonHelen K JohnstonAurelia DefourAditi PhadkeQing YuJyoti K JaiswalKanneboyina NagarajuPublished in: Skeletal muscle (2020)
Our results provide evidence that calpain-3 deficiency in the skeletal muscle is associated with poor mitochondrial biogenesis and function resulting in poor sarcolemmal repair. Addressing this deficit by drugs that improve mitochondrial activity offers new therapeutic avenues for LGMD2A.