Absence of dysferlin alters myogenin expression and delays human muscle differentiation “in vitro”

N De Luna, E Gallardo, M Soriano… - Journal of Biological …, 2006 - ASBMB
Mutations in dysferlin cause a type of muscular dystrophy known as dysferlinopathy.
Dysferlin may be involved in muscle repair and differentiation. We compared normal human
skeletal muscle cultures expressing dysferlin with muscle cultures from dysferlinopathy
patients. We quantified the fusion index of myoblasts as a measure of muscle development
and conducted optic and electronic microscopy, immunofluorescence, Western blot, flow
cytometry, and real-time PCR at different developmental stages. Short interference RNA was …