ATP synthase F₀ complex subunit a, mitochondrially encoded (MT-ATP6)

MT-ATP6 is a 226-amino acid, mitochondrially encoded integral membrane protein forming part of the F₀ sector of the ATP synthase complex (complex V) in the mitochondrial inner membrane[1][2]. It is responsible for facilitating proton translocation together with subunit c, forming the core of the mitochondrial proton channel required for ATP production[3][5]. The proton gradient generated by upstream electron transport complexes is utilized by ATP synthase via the rotary mechanism of the F₁ and F₀ domains, converting ADP and phosphate to ATP—the universal energy currency of the cell[1][2][3]. Pathogenic mutations in MT-ATP6 impair ATP generation and are strongly linked to Leigh syndrome and other mitochondrial encephalopathies[4]. Pharmacologic inhibition of ATP synthase F₀ (e.g., by oligomycin) blocks ATP synthesis and can be lethal due to failure of energy conversion in all tissues[1][5][4].

Other names

ATP6ATPASE6MTATP6ATPase-6Su6mF-ATPase protein 6Proton-conducting channelATP synthase membrane subunit 6ATP synthase F₀ complex subunit a

Inhibition of proton flow (oligomycin, DCCD block the F₀ channel) Impairment of ATP synthesis (energy production blockade) Uncoupling of proton gradient and ATP production