Atp5f1a - ATP synthase, H+ transporting, mitochondrial F1 complex, alpha subunit 1

Enables ATP binding activity and proton-transporting ATP synthase activity, rotational mechanism. Acts upstream of or within lipid metabolic process. Located in membrane and mitochondrion. Is expressed in several structures, including alimentary system; integumental system; nervous system; respiratory system; and sensory organ. Human ortholog(s) of this gene implicated in combined oxidative phosphorylation deficiency 22 and mitochondrial complex V (ATP synthase) deficiency nuclear type 4. Orthologous to human ATP5F1A (ATP synthase F1 subunit alpha). [provided by Alliance of Genome Resources, Apr 2022]