Specifications
Immunogen
A synthetic peptide of human ATP6V1B1 is used for rabbit immunization.
Target
Alternative Names
ATP6V1B1; ATPase, H+ transporting, lysosomal 56/58kDa, V1 subunit B1; ATP6B1, vacuolar proton pump 3 , VPP3; V-type proton ATPase subunit B, kidney isoform; Renal tubular acidosis with deafness; RTA1B; VATB; Vma2; V-ATPase B1 subunit; V-ATPase subunit B 1
Product Background
Antigen Description
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.
Pathway
Collecting duct acid secretion, organism-specific biosystem; Collecting duct acid secretion, conserved biosystem; Epithelial cell signaling in Helicobacter pylori infection, organism-specific biosystem; Epithelial cell signaling in Helicobacter pylori infection, conserved biosystem; Insulin receptor recycling, organism-specific biosystem; Iron uptake and transport, organism-specific biosystem; Metabolic pathways, organism-specific biosystem;
Citations
Publication ()
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