Vacuolar-type H+-ATPase (V-ATPase) is a multisubunit enzyme responsible for acidification of eukaryotic intracellular organelles. V-ATPases pump protons against an electrochemical gradient, while F-ATPases reverse the process, thereby synthesizing ATP. A peripheral V1 domain, which is responsible for ATP hydrolysis, and a integral V0 domain, which is responsible for proton translocation, compose V-ATPase. Nine subunits (A-H) make up the V1 domain and five subunits (a, d, c, c' and c") make up the V0 domain. Like F-ATPase, V-ATPase most likely operates through a rotary mechanism. The V-ATPase V1 B subunit exists as two isoforms. In the inner ear, the V-ATPase B1 isoform functions in proton secretion and is required to maintain proper endolymph pH and normal auditory function. The gene encoding the human V-ATPase B1 isoform maps to chromosome 2cen-q13. Mutations in this gene cause distal renal tubular acidosis associated with sensorineural deafness. The V-ATPase B2 isoform is expressed in kidney and is the only B isoform expressed in osteoclasts.
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Protein Aliases: ATPase, H+ transporting, lysosomal (vacuolar proton pump), beta 56/58 kDa, isoform 1; ATPase, H+ transporting, lysosomal 56/58kDa, V1 subunit B, isoform 1; ATPase, H+ transporting, lysosomal 56/58kDa, V1 subunit B1; ATPase, H+ transporting, V1 subunit B, isoform 1; Endomembrane proton pump 58 kDa subunit; H(+)-transporting two-sector ATPase, 58kD subunit; H+-ATPase beta 1 subunit; lysosomal 56/58kDa; V-ATPase B1; V-ATPase B1 subunit; V-ATPase subunit B 1; V-type proton ATPase subunit B, kidney isoform; vacuolar proton pump 3; Vacuolar proton pump subunit B 1; vacuolar proton pump, subunit 3
Gene Aliases: ATP6B1; ATP6V1B1; AW208839; D630003L15; D630030L16Rik; D630039P21Rik; RTA1B; VATB; VMA2; Vpp-3; VPP3
UniProt ID: (Human) P15313
Molecular Function: ATP synthase