Ontology highlight
ABSTRACT:
SUBMITTER: Demonbreun AR
PROVIDER: S-EPMC3873498 | biostudies-other | 2014 Jan
REPOSITORIES: biostudies-other
Demonbreun Alexis R AR Rossi Ann E AE Alvarez Manuel G MG Swanson Kaitlin E KE Deveaux H Kieran HK Earley Judy U JU Hadhazy Michele M Vohra Ravneet R Walter Glenn A GA Pytel Peter P McNally Elizabeth M EM
The American journal of pathology 20131029 1
Dysferlin is a membrane-associated protein implicated in muscular dystrophy and vesicle movement and function in muscles. The precise role of dysferlin has been debated, partly because of the mild phenotype in dysferlin-null mice (Dysf). We bred Dysf mice to mice lacking myoferlin (MKO) to generate mice lacking both myoferlin and dysferlin (FER). FER animals displayed progressive muscle damage with myofiber necrosis, internalized nuclei, and, at older ages, chronic remodeling and increasing crea ...[more]