Rabbit anti-Human ATP6V1B1 Polyclonal Antibody | anti-ATP6V1B1 antibody

ATP6V1B1, CT (ATP6V1B1, ATP6B1, VATB, VPP3, V-type proton ATPase subunit B, kidney isoform, Endomembrane proton pump 58kD subunit, Vacuolar proton pump subunit B 1) (Biotin)

Cat. Num. AAA6276669

• Gene Names: ATP6V1B1; VATB; VMA2; VPP3; RTA1B; ATP6B1
• Reactivity: Human
• Applications: Immunohistochemistry, ELISA, Western Blot
• Purity: Purified by Protein A Affinity Chromatography.
• Synonyms: ATP6V1B1; Polyclonal Antibody; CT (ATP6V1B1; ATP6B1; VATB; VPP3; V-type proton ATPase subunit B; kidney isoform; Endomembrane proton pump 58kD subunit; Vacuolar proton pump subunit B 1) (Biotin); Vacuolar proton pump subunit B 1; anti-ATP6V1B1 antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Isotype: IgG
• Purity/Purification: Purified by Protein A Affinity Chromatography.
• Form/Format: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Biotin.

• Applicable Applications for anti-ATP6V1B1 antibody: Immunohistochemistry (IHC), ELISA (EIA), Western Blot (WB)
• Application Notes: IHC: 1:10-50; ELISA: 1:1,000; WB: 1:100-500; Applications are based on unconjugated antibody.
• Immunogen: ATP6V1B1 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 471-501 amino acids from the C-terminal region of human ATP6V1B1.
• Conjugate: Biotin
• Note: Preservative Free
• Preparation and Storage: Store product at 4 degree C if to be used immediately within two weeks. For long-term storage, aliquot to avoid repeated freezing and thawing and store at -20 degree C. Aliquots are stable at -20 degree C for 12 months after receipt. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Western Blot (WB)

(ATP6V1B1 Antibody (C-term) western blot analysis in 293 cell line lysates (35ug/lane).This demonstrates the ATP6V1B1 antibody detected the ATP6V1B1 protein (arrow).)

Immunohistochemistry (IHC)

(ATP6V1B1 Antibody (C-term) immunohistochemistry analysis in formalin fixed and paraffin embedded human breast carcinoma followed by peroxidase conjugation of the secondary antibody and DAB staining.This data demonstrates the use of ATP6V1B1 Antibody (C-term) for immunohistochemistry. Clinical relevance has not been evaluated.)

Product Categories/Family for anti-ATP6V1B1 antibody

Antibodies; Enzymes; ATPase

References

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).

NCBI and Uniprot Product Information

• NCBI GI #: 19913426
• NCBI GeneID: 525
• NCBI Accession #: NP_001683.2
• 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; V-ATPase B1 subunit; V-ATPase subunit B 1; vacuolar proton pump 3; H+-ATPase beta 1 subunit; vacuolar proton pump, subunit 3; vacuolar proton pump subunit B 1; endomembrane proton pump 58 kDa subunit; H(+)-t
• 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