|Other Names||V-type proton ATPase subunit B, kidney isoform, V-ATPase subunit B 1, Endomembrane proton pump 58 kDa subunit, Vacuolar proton pump subunit B 1, ATP6V1B1, ATP6B1, VATB, VPP3|
|Format||Synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml DI water for a final concentration of 1 mg/ml.|
|Storage||Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C.|
|Precautions||This product is for research use only. Not for use in diagnostic or therapeutic procedures.|
|Synonyms||ATP6B1, VATB, VPP3|
|Function||Non-catalytic subunit of the peripheral V1 complex of vacuolar ATPase. V-ATPase is responsible for acidifying a variety of intracellular compartments in eukaryotic cells.|
|Cellular Location||Endomembrane system; Peripheral membrane protein. Note=Endomembrane|
|Tissue Location||Expressed in the cochlea and endolymphatic sac.|
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This gene encodes a component of vacuolar ATPase(V-ATPase), a multisubunit enzyme that mediates acidification ofeukaryotic intracellular organelles. V-ATPase dependent organelleacidification is necessary for such intracellular processes asprotein sorting, zymogen activation, receptor-mediated endocytosis,and synaptic vesicle proton gradient generation. V-ATPase iscomposed of a cytosolic V1 domain and a transmembrane V0 domain.The V1 domain consists of three A and three B subunits, two Gsubunits plus the C, D, E, F, and H subunits. The V1 domaincontains the ATP catalytic site. The V0 domain consists of fivedifferent subunits: a, c, c', c'', and d. Additional isoforms ofmany of the V1 and V0 subunit proteins are encoded by multiplegenes or alternatively spliced transcript variants. This encodedprotein is one of two V1 domain B subunit isoforms and is found inthe kidney. Mutations in this gene cause distal renal tubularacidosis associated with sensorineural deafness. [provided byRefSeq].
Bailey, S.D., et al. Diabetes Care 33(10):2250-2253(2010)Sharifian, M., et al. Iran J Kidney Dis 4(3):202-206(2010)Talmud, P.J., et al. Am. J. Hum. Genet. 85(5):628-642(2009)Andreucci, E., et al. Pediatr. Nephrol. 24(11):2147-2153(2009)Sethi, S.K., et al. Indian Pediatr 46(5):425-427(2009)
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