Mouse ATP6V1B1 Monoclonal Antibody | anti-ATP6V1B1 antibody

ATP6V1B1 antibody

Cat. Num. AAA831242

• Gene Names: ATP6V1B1; VATB; VMA2; VPP3; RTA1B; ATP6B1
• Applications: Flow Cytometry, Functional Assay, Immunofluorescence, Western Blot
• Purity: ATP6V1B1 antibody was purified by affinity chromatography.
• Synonyms: ATP6V1B1; Monoclonal Antibody; ATP6V1B1 antibody; Monoclonal ATP6V1B1; Anti-ATP6V1B1; Atpase H+ Transporting Lysosomal 56/58Kda V1 Subunit B1; ATP6B1; MGC32642; RTA1B; VATB; VMA2; VPP3; ATPV1B1 6; ATPV1B1-6; anti-ATP6V1B1 antibody

• Host: Mouse
• Clonality: Monoclonal
• Isotype: IgG1
• Clone Number: 6D2
• Purity/Purification: ATP6V1B1 antibody was purified by affinity chromatography.
• Form/Format: Supplied in PBS buffer, pH 7.3, containing 1% BSA, 50% glycerol and 0.02% sodium azide.
• Concentration: 500 ug-1 mg/ml (varies by lot)
• Sequence Length: 513

• Applicable Applications for anti-ATP6V1B1 antibody: Flow Cytometry (FC/FACS), Immunofluorescence (IF), Western Blot (WB)
• Application Notes: FC: 1:100; IF: 1:100; WB: 1:2000
• Biological Significance: ATP6V1B1 is a component of vacuolar ATPase (V-ATPase), a multisubunit enzyme that mediates acidification ofeukaryotic 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 ofthree A and three B subunits, two G subunits plus the C, D, E, F, and H subunits. The V1 domain contains the ATPcatalytic site. The V0 domain consists of five different subunits: a, c, c', c'', and d.
• Cross-Reactivity: Human
• Immunogen: ATP6V1B1 antibody was raised in mouse using a full length recombinant protein of human ATP6V1B1 (NP_001683) produced in HEK293T cells, as the immunogen.
• Preparation and Storage: Store at 4 degree C for short term storage. Aliquot and store at -20 degree C for long term storage. Avoid repeated freeze/thaw cycles.

Western Blot (WB)

(Western Blot analysis of HEK293T cell lysates (5 ug) transfected with either recombinant ATP6V1B1 protein (Right) or empty vector (Left) detected with ATP6V1B1 antibody)

Immunofluorescence (IF)

(Immunofluorescent staining of COS7 cells transiently transfected with recombinant ATP6V1B1 protein using ATP6V1B1 antibody)

Related Product Information for anti-ATP6V1B1 antibody

Mouse monoclonal ATP6V1B1 antibody

Product Categories/Family for anti-ATP6V1B1 antibody

Proteases; Inhibitors; & Enzymes

NCBI and Uniprot Product Information

• NCBI GI #: 19913426
• NCBI GeneID: 525
• NCBI Accession #: NP_001683
• NCBI GenBank Nucleotide #: NM_001692.3
• UniProt Accession #: P15313; Q53FY0; Q6P4H6
• Molecular Weight: 56,833 Da
• NCBI Official Full Name: V-type proton ATPase subunit B, kidney isoform
• NCBI Official Synonym Full Names: ATPase, H+ transporting, lysosomal 56/58kDa, 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: EC 3.6.3.14; Energy Metabolism - oxidative phosphorylation; Hydrolase

Chromosomal Location of Human Ortholog: 2p13.1

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

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

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

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