ATPase, H+ transporting, lysosomal V0 subunit a4


ATP6V0A4 (may also be known as: None)




This gene encodes a component of vacuolar ATPase (V-ATPase), a multisubunit enzyme that mediates acidification of intracellular compartments of eukaryotic cells. V-ATPase dependent acidification is necessary for such intracellular processes as protein sorting, zymogen activation, receptor-mediated endocytosis, and synaptic vesicle proton gradient generation. V-ATPase is composed of a cytosolic V1 domain and a transmembrane V0 domain. The V1 domain consists of three A and three B subunits, two G subunits plus the C, D, E, F, and H subunits. The V1 domain contains the ATP catalytic site. The V0 domain consists of five different subunits: a, c, c', c'', and d. This gene is one of four genes in man and mouse that encode different isoforms of the a subunit. Alternatively spliced transcript variants encoding the same protein have been described. Mutations in this gene are associated with renal tubular acidosis associated with preserved hearing. [provided by RefSeq, Jul 2008]


unc-32 Caenorhabditis elegans
Vha100-1 Drosophila melanogaster
Atp6v0a4 Mus musculus
Atp6v0a4 Rattus norvegicus

Links to external resources

Changes associated with this gene

GO Terms

GO IDGO TermGO Category
GO:0001503 ossification biological_process
GO:0006811 ion transport biological_process
GO:0006879 cellular iron ion homeostasis biological_process
GO:0006885 regulation of pH biological_process
GO:0007588 excretion biological_process
GO:0007605 sensory perception of sound biological_process
GO:0008286 insulin receptor signaling pathway biological_process
GO:0015991 ATP hydrolysis coupled proton transport biological_process
GO:0015992 proton transport biological_process
GO:0033572 transferrin transport biological_process
GO:0055085 transmembrane transport biological_process
GO:0005768 endosome cellular_component
GO:0005886 plasma membrane cellular_component
GO:0010008 endosome membrane cellular_component
GO:0016021 integral to membrane cellular_component
GO:0016324 apical plasma membrane cellular_component
GO:0016471 vacuolar proton-transporting V-type ATPase complex cellular_component
GO:0031526 brush border membrane cellular_component
GO:0033177 proton-transporting two-sector ATPase complex, proton-transporting domain cellular_component
GO:0045177 apical part of cell cellular_component
GO:0005515 protein binding molecular_function
GO:0015078 hydrogen ion transmembrane transporter activity molecular_function
GO:0051117 ATPase binding molecular_function