Rabbit anti-Human ATP6V1B1 Polyclonal Antibody

Uniprot Id

P15313

Target Species

Human

Target Name

ATP6V1B1

Target Full Name

V-type proton ATPase subunit B, kidney isoform

Target Function

Non-catalytic subunit of the V1 complex of vacuolar(H+)-ATPase (V-ATPase), a multisubunit enzyme composed of a peripheral complex (V1) that hydrolyzes ATP and a membrane integral complex (V0) that translocates protons. V-ATPase is responsible for acidifying and maintaining the pH of intracellular compartments and in some cell types, is targeted to the plasma membrane, where it is responsible for acidifying the extracellular environment. Essential for the proper assembly and activity of V-ATPase. In renal intercalated cells, mediates secretion of protons (H+) into the urine thereby ensuring correct urinary acidification. Required for optimal olfactory function by mediating the acidification of the nasal olfactory epithelium.

Target Involvement

Renal tubular acidosis, distal, with progressive nerve deafness (dRTA-D)

Target Subcellular Location

Apical cell membrane. Basolateral cell membrane.

Target Protein Families

ATPase alpha/beta chains family

Target Tissue Specificity

Kidney; localizes to early distal nephron, encompassing thick ascending limbs and distal convoluted tubules (at protein level). Expressed in the cochlea and endolymphatic sac.

Target Synonyms

Atp6b1; ATP6V1B1; 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; V1 subunit B; isoform 1; Endomembrane proton pump 58 kDa subunit; H(+) transporting two sector ATPase 58kD subunit; H+ ATPase beta 1 subunit; kidney isoform; Lysosomal 56/58kDa; MGC32642; RTA1B; V ATPase B1 subunit; V type proton ATPase subunit B kidney isoform; V type proton ATPase subunit B; kidney isoform; V-ATPase subunit B 1; V-type proton ATPase subunit B; Vacuolar proton pump 3; Vacuolar proton pump subunit 3; Vacuolar proton pump subunit B 1; VATB; VATB1_HUMAN; VMA2; Vpp 3; Vpp3

Target Background

This gene encodes a component of vacuolar ATPase (V-ATPase), a multisubunit enzyme that mediates acidification of eukaryotic intracellular organelles. V-ATPase dependent organelle 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. Additional isoforms of many of the V1 and V0 subunit proteins are encoded by multiple genes or alternatively spliced transcript variants. This encoded protein is one of two V1 domain B subunit isoforms and is found in the kidney. Mutations in this gene cause distal renal tubular acidosis associated with sensorineural deafness.