Defective mitochondrial fusion causes severe skeletal muscle degeneration in double mitofusin 1/2 knockout mice

Mitochondrial fusion is a critical process involved in the maintenance of mitochondrial bioenergetics, shape and number. This event is mainly orchestrated by two GTPases termed mitofusins 1 and 2 (MFN1/2). Considering that skeletal muscle is the tissue with the highest metabolic demand in our body, we tested here the consequences of intrinsic defective mitochondrial fusion in skeletal muscle physiology.