Autophagy is defective in collagen VI muscular dystrophies, and its reactivation rescues myofiber degeneration

Autophagy is often believed to be elevated in disease, contributing to pathogenesis. Paolo Bonaldo and his colleagues now show that it is actually too little autophagy that occurs in some forms of muscular dystrophy, resulting in the continued presence of defective mitochondria and thus myofiber degeneration. They also show that increasing autophagy via dietary or pharmacological means can ameliorate muscle pathology in a mouse model of human muscular dystrophy.