Cardiomyopathic features associated with muscular dystrophy are independent of dystrophin absence in cardiovasculature

TA Hainsey, S Senapati, DE Kuhn, JA Rafael - Neuromuscular Disorders, 2003 - Elsevier
The loss of dystrophin results in skeletal muscle degeneration and cardiomyopathy in
patients with Duchenne muscular dystrophy. In skeletal muscle, dystrophin strengthens the
myofiber membrane by linking the submembranous cytoskeleton and extracellular matrix
through its direct interaction with the dystroglycan/sarcoglycan complex. In limb-girdle
muscular dystrophy, the loss of the sarcoglycans in cardiovasculature leads to
cardiomyopathy. It is unknown whether the absence of dystrophin in cardiomyocytes or …