Progress of mitofusin-2 in mitochondrial homeostasis in skeletal muscle / 国际儿科学杂志

ABSTRACT

Mitochondria homeostasis is essential to cell activities,while mitochondrial dysfunction is involved in the pathogenesis of many muscular diseases,such as congenital myopathy and muscular dystrophy.However,its detailed mechanism has not been clarified.Mitofusin-2 (Mfn2) is located in the outer mitochondrial membranes and endoplasmic reticulum(ER) mitochondrial contact sites.Recently,researchers have found that in addition to its participation in tethering mitochondiia to mitochondria and ER to mitochondria,Mfn2 regulates mitochondrial dynamics and multiple cellular processes including oxidative stress,Ca2 + signaling pathways,energy metabolism,and PINK1/Parkin induced mitophagy,playing an important role in maintaining the mitochondrial quality control.And abnormal expression of Mfn2 also related to many kinds of pathological states.Here we review its molecular structure and recent advances in the role of Mfn2 in maintaining mitochondrial homeostasis in skeletal muscle.