Transcriptional and histopathological profiling of skeletal muscle in Bla/J mice at the stage of dysferlinopathy manifestation.
It is demonstrated that early muscle atrophy and myofiber necrosis in dysferlinopathy occur independently of canonical ubiquitin-proteasome and apoptotic pathways, and the disease manifestation stage is structurally characterized by stalled mitochondrial clearance and a delayed regenerative response.