MPV17 mitochondrial inner membrane protein (MPV17)

MPV17 is a small integral protein (18 kDa, 176 amino acids) localized to the inner mitochondrial membrane, where it forms a non-selective channel with gating properties sensitive to membrane potential, pH, and redox state[5][3]. Its main function is to preserve *mitochondrial homeostasis* by regulating membrane potential, controlling reactive oxygen species production, and supporting the maintenance of mitochondrial DNA integrity[3][1][2]. Mutations in MPV17 destabilize the protein and disrupt channel function, leading to diseases such as mitochondrial DNA depletion syndrome, with symptoms dependent on tissue type—often impacting the liver, brain, and kidneys[2][1][5]. MPV17 interacts physically with several mitochondrial proteins, including ATP synthase, Cyclophilin D, MIC60 (mitofilin), and GRP75, influencing mitochondrial cristae structure and calcium homeostasis[1]. It has context-dependent effects on apoptosis: protective in most cell types, but can promote beta-cell death in pancreatic islets under diabetic conditions[4]. MPV17 is not an established therapeutic target, and there are currently no approved drugs that directly act on this protein[1][2][3][4][5].

Other names

MPV17 (canonical abbreviation)MPV17 geneMPV17 proteinMitochondrial inner membrane protein MPV17Sym1 (yeast ortholog)