Recombinant Human ATP synthase subunit a (MT-ATP6), Truncated

Uniprot Id

P00846

Target Species

Human

Target Name

MT-ATP6

Target Full Name

ATP synthase subunit a

Target Function

Mitochondrial membrane ATP synthase (F(1)F(0) ATP synthase or Complex V) produces ATP from ADP in the presence of a proton gradient across the membrane which is generated by electron transport complexes of the respiratory chain. F-type ATPases consist of two structural domains, F(1) - containing the extramembraneous catalytic core and F(0) - containing the membrane proton channel, linked together by a central stalk and a peripheral stalk. During catalysis, ATP synthesis in the catalytic domain of F(1) is coupled via a rotary mechanism of the central stalk subunits to proton translocation. Key component of the proton channel; it may play a direct role in the translocation of protons across the membrane

Target Involvement

Neuropathy, ataxia, and retinitis pigmentosa (NARP); Leber hereditary optic neuropathy (LHON); Leigh syndrome (LS); Mitochondrial infantile bilateral striatal necrosis (MIBSN); Mitochondrial complex V deficiency, mitochondrial 1 (MC5DM1); Myopathy, lactic acidosis, and sideroblastic anemia 3 (MLASA3); Ataxia and polyneuropathy, adult-onset (APAO); Cardiomyopathy, infantile hypertrophic (CMHI)

Target Subcellular Location

Mitochondrion inner membrane; Multi-pass membrane protein.

Target Protein Families

ATPase A chain family

Target Synonyms

MT-ATP6; ATP6; ATPASE6; MTATP6; ATP synthase subunit a; F-ATPase protein 6