Disorders of autophagy – the degradation of damaged cellular organelles – are associated with muscular dystrophy, or muscle wasting, according to scientists from Italy in the journal Nature Medicine.
Autophagy is a key mechanism for the breakdown of damaged cellular organelles, and abnormalities in this process underlie many diseases. Until now, little was known about autophagy in patients with muscular dystrophy. Previous research has indicated that some forms of muscular dystrophy exhibit abnormal mitochondria and spontaneous cell death – leading to muscle degeneration.
Paolo Bonaldo and colleagues from the University of Padua studied mice with congenital muscular dystrophy (caused by a mutation) and showed that the presence of abnormal mitochondria and cell death are caused by disorders of autophagy in muscle cells.
The researchers also showed that forced activation of the autophagy process in muscle cells ensures their survival – suggesting that it could treat patients with muscle wasting. (PAP)