Myogenesis in dysferlin-deficient myoblasts is inhibited by an intrinsic inflammatory response
TV Cohen, JE Cohen, TA Partridge - Neuromuscular Disorders, 2012 - Elsevier
Limb-girdle muscular dystrophy type 2B results from mutations in dysferlin, a membrane-
associated protein involved in cellular membrane repair. Primary myoblast cultures derived
from dysferlinopathy patients show reduced myogenic potential, suggesting that dysferlin
may regulate myotube fusion and be required for muscle regeneration. These observations
contrast with the findings that muscle develops normally in pre-symptomatic dysferlinopathy
patients. To better understand the role of dysferlin in myogenesis, we investigated this …
associated protein involved in cellular membrane repair. Primary myoblast cultures derived
from dysferlinopathy patients show reduced myogenic potential, suggesting that dysferlin
may regulate myotube fusion and be required for muscle regeneration. These observations
contrast with the findings that muscle develops normally in pre-symptomatic dysferlinopathy
patients. To better understand the role of dysferlin in myogenesis, we investigated this …