Skeletal, cardiac, and respiratory muscle function and histopathology in the P448Lneo- mouse model of FKRP-deficient muscular dystrophy.

Qing YuMelissa MoralesNing LiAlexander G FritzRen RuobingAnthony BlaeserErshia FrancoisQi-Long LuKanneboyina NagarajuChristopher F Spurney

Published in: Skeletal muscle (2018)

FKRP-deficient P448Lneo- mice demonstrate significant deficits in cardiac and respiratory functions compared to control mice, and this is associated with increased inflammation and fibrosis. This study provides new functional outcome measures for preclinical trials of FKRP-related muscular dystrophies.

Keyphrases

muscular dystrophy
mouse model
duchenne muscular dystrophy
wild type
high fat diet induced
left ventricular
oxidative stress
traumatic brain injury
type diabetes
insulin resistance
metabolic syndrome
resistance training
atrial fibrillation