Monoamine oxidase inhibition prevents mitochondrial dysfunction and apoptosis in myoblasts from patients with collagen VI myopathies

Scheme of the pathway leading from Collagen VI deficiency to loss of cell viability through MAO-dependent ROS formation. The present study in human myoblasts demonstrated the role of MAO in oxidative stress and mitochondrial dysfunction confirming evidence obtained previously in mouse models of muscular dystrophy. In those models we documented also the oxidation of myofibrillar proteins that are involved in contractile impairment of viable myocites.110