CPRiL

ATP6V0A4

ATPase H+ transporting V0 subunit a4

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]

• V-type proton ATPase 116 kDa subunit a 4
• ATPase, H+ transporting, lysosomal (vacuolar proton pump) non-catalytic accessory protein 1B
• ATPase, H+ transporting, lysosomal (vacuolar proton pump) non-catalytic accessory protein 2 (38kD)
ATPase, H+ transporting, lysosomal V0 subunit a4
H(+)-transporting two-sector ATPase, noncatalytic accessory protein 1B
V-ATPase 116 kDa
V-ATPase 116 kDa isoform a 4
V-ATPase subunit a4
V-type proton ATPase 116 kDa subunit a
vacuolar proton pump 116 kDa accessory subunit
vacuolar proton pump, subunit 2
A4
ATP6N1B
ATP6N2
DRTA3
RDRTA2
RTA1C
RTADR
STV1
VPH1
VPP2