Rabbit ATP6V1B1 Polyclonal Antibody | anti-ATP6V1B1 antibody

ATP6V1B1 Polyclonal Antibody

Cat. Num. AAA3006288

• Gene Names: ATP6V1B1; VATB; VMA2; VPP3; RTA1B; ATP6B1
• Reactivity: Human, Mouse, Rat
• Applications: Western Blot, Immunohistochemistry
• Purity: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is >95% (by SDS-PAGE).
• Synonyms: ATP6V1B1; Polyclonal Antibody; ATP6V1B1 Polyclonal Antibody; 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; ATP6B1; VATB; VPP3; V-ATPase B1; anti-ATP6V1B1 antibody

• Host: Rabbit
• Reactivity: Human, Mouse, Rat
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: ATP6V1B1 polyclonal antibody detects endogenous levels of ATP6V1B1 protein.
• Purity/Purification: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is >95% (by SDS-PAGE).
• Form/Format: Rabbit IgG, 1mg/ml in PBS with 0.02% sodium azide, 50% glycerol, pH7.2
• Sequence Length: 513

• Applicable Applications for anti-ATP6V1B1 antibody: Western Blot (WB), Immunohistochemistry (IHC)
• Application Notes: WB: 1:500-1:1000; IHC: 1:50-1:200
• Immunogen: Synthetic peptide, corresponding to amino acids 426-471 of Human ATP6V1B1.
• Preparation and Storage: Store at 4 degree C short term.; Aliquot and store at -20 degree C long term.; Avoid freeze-thaw cycles.

Western Blot (WB)

(Western Blot (WB) analysis of ATP6V1B1 polyclonal antibody at 1:500 dilutionLane1:HEK293T cell lysateLane2:Raw264. 7 cell lysateLane3:PC12 cell lysate)

Related Product Information for anti-ATP6V1B1 antibody

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.

NCBI and Uniprot Product Information

• NCBI GI #: 19913426
• NCBI GeneID: 525
• NCBI Accession #: NP_001683.2
• NCBI GenBank Nucleotide #: NP_001683.2
• UniProt Accession #: P15313
• Molecular Weight: ~ 58kDa
• NCBI Official Full Name: V-type proton ATPase subunit B, kidney isoform
• NCBI Official Synonym Full Names: ATPase H+ transporting V1 subunit B1
• NCBI Official Symbol: ATP6V1B1
• NCBI Official Synonym Symbols: VATB; VMA2; VPP3; RTA1B; ATP6B1
• NCBI Protein Information: V-type proton ATPase subunit B, kidney isoform
• UniProt Protein Name: V-type proton ATPase subunit B, kidney isoform
• Protein Family: V-type proton ATPase
• UniProt Gene Name: ATP6V1B1
• UniProt Synonym Gene Names: ATP6B1; VATB; VPP3; V-ATPase subunit B 1
• UniProt Entry Name: VATB1_HUMAN

NCBI 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. [provided by RefSeq, Jul 2008]

Uniprot Description

ATP6V1B1: 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. Defects in ATP6V1B1 are the cause of distal renal tubular acidosis with deafness (dRTA-D). Inheritance is autosomal recessive. Patients with recessive dRTA are severely affected, presenting with either acute illness or growth failure at a young age, and bilateral sensorineural deafness. Other features include low serum K(+) due to renal potassium wasting, and elevated urinary calcium. If untreated, this acidosis may result in dissolution of bone, leading to osteomalacia and rickets. Renal deposition of calcium salts (nephrocalcinosis) and renal stone formation commonly occur. Belongs to the ATPase alpha/beta chains family.

Protein type: Hydrolase; EC 3.6.3.14; Energy Metabolism - oxidative phosphorylation

Chromosomal Location of Human Ortholog: 2p13.1

Cellular Component: microvillus; basolateral plasma membrane; apical plasma membrane; cytoplasm; endomembrane system; vacuolar proton-transporting V-type ATPase complex; cytosol; lateral plasma membrane

Molecular Function: protein complex binding; hydrogen ion transmembrane transporter activity; hydrolase activity, acting on acid anhydrides, catalyzing transmembrane movement of substances; ATP binding

Biological Process: interaction with host; inner ear morphogenesis; ossification; cellular iron ion homeostasis; transferrin transport; excretion; calcium ion homeostasis; ATP metabolic process; proton transport; sensory perception of sound; ATP hydrolysis coupled proton transport; pH reduction; insulin receptor signaling pathway; regulation of pH; transmembrane transport

Disease: Renal Tubular Acidosis, Distal, With Progressive Nerve Deafness