The role of mitochondria in Duchenne muscular dystrophy.

Abstract

Duchenne muscular dystrophy (DMD) is an X-linked lethal disorder caused by mutations in the dystrophin gene. Progression of this disease may lead to cardiomyopathy and respiratory failure, which are the main causes of death among DMD patients. Lack of dystrophin affects cellular myogenic function and related organelles. Dystrophin deficiency results in intracellular Ca2+ dysregulation, mitochondrial dysfunction and induces elevated production of reactive oxygen species (ROS). Due to current findings, mitochondria may be also a potential target for DMD therapy. In this review we attempted to provide an insight into the role of mitochondria in perpetuation of DMD disease.