Mitofusin-2 is as essential transmembrane GTPase which mediates mitochondrial fusion. Fusion of mitochondria occurs in many cell types and constitutes an important step in mitochondria morphology, which is balanced between fusion and fission. Mitofusin-2 acts independently of the cytoskeleton,Porcinelays a central role in mitochondrial metabolism, and is associated with obesity and/or apoptosis processes. Defects in mitofusin-2 are the cause of Charcot-Marie-Tooth disease type 2A2 (CMT2A2). CMT2A2 is a form of Charcot-Marie-Tooth disease, the most common inherited disorder of the peripheral nervous system.