ATP5A1 Antibody – Mouse Monoclonal

This gene encodes a subunit of mitochondrial ATP synthase. Mitochondrial ATP synthase catalyzes ATP synthesis, using an electrochemical gradient of protons across the inner membrane during oxidative phosphorylation. ATP synthase is composed of two linked multi-subunit complexes: the soluble catalytic core, F1, and the membrane-spanning component, Fo, comprising the proton channel. The catalytic portion of mitochondrial ATP synthase consists of 5 different subunits (alpha, beta, gamma, delta, and epsilon) assembled with a stoichiometry of 3 alpha, 3 beta, and a single representative of the other 3. The proton channel consists of three main subunits (a, b, c). This gene encodes the alpha subunit of the catalytic core. Alternatively spliced transcript variants encoding the different isoforms have been identified. Pseudogenes of this gene are located on chromosomes 9, 2, and 16. [provided by RefSeq, Mar 2012]

ATP5A1: 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. Subunits alpha and beta form the catalytic core in F(1). Rotation of the central stalk against the surrounding alpha(3)beta(3) subunits leads to hydrolysis of ATP in three separate catalytic sites on the beta subunits. Subunit alpha does not bear the catalytic high-affinity ATP-binding sites. Belongs to the ATPase alpha/beta chains family.

Protein type: Transporter; Hydrolase; Membrane protein, integral; EC 3.6.3.14; Mitochondrial; Energy Metabolism - oxidative phosphorylation

Chromosomal Location of Human Ortholog: 18q21

Cellular Component: membrane; mitochondrial inner membrane; mitochondrial matrix; mitochondrial proton-transporting ATP synthase complex; mitochondrion; myelin sheath; plasma membrane; proton-transporting ATP synthase complex, catalytic core F(1); signalosome

Molecular Function: ATP binding; ATPase activity; hydrogen ion transporting ATP synthase activity, rotational mechanism; hydrogen ion transporting ATPase activity, rotational mechanism; MHC class I protein binding; protein binding; transmembrane transporter activity

Biological Process: ATP biosynthetic process; ATP hydrolysis coupled proton transport; embryonic development; lipid metabolic process; mitochondrial ATP synthesis coupled proton transport; negative regulation of endothelial cell proliferation

Disease: Combined Oxidative Phosphorylation Deficiency 22; Mitochondrial Complex V (atp Synthase) Deficiency, Nuclear Type 4