Rabbit anti-Human ATP6V0A4 Polyclonal Antibody | anti-ATP6V0A4 antibody

Anti-ATP6V0A4 Antibody (aa53-295) IHC-plus

Cat. Num. AAA249881

• Gene Names: ATP6V0A4; A4; STV1; VPH1; VPP2; RTA1C; RTADR; ATP6N2; RDRTA2; ATP6N1B
• Reactivity: Human
• Applications: Immunohistochemistry, Western Blot
• Purity: Immunoaffinity purified
• Synonyms: ATP6V0A4; Polyclonal Antibody; Anti-ATP6V0A4 Antibody (aa53-295) IHC-plus; Rabbit Polyclonal (IgG) to Human ATP6V0A4; Human ATP6V0A4; ATP6N1B; Renal tubular acidosis; RTA1C; STV1; V-ATPase 116 kDa; V-ATPase alpha 4; RTADR; ATP6N2; RDRTA2; V-ATPase 116 kDa isoform a4; VPH1; VPP2; anti-ATP6V0A4 antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: Human ATP6V0A4
• Purity/Purification: Immunoaffinity purified
• Form/Format: 0.1 M Tris-glycine, pH 7.0, 10% glycerol, 0.01% Thimerosal
• Concentration: 1 mg/ml (varies by lot)
• Sequence Length: 840

• Applicable Applications for anti-ATP6V0A4 antibody: Immunohistochemistry (IHC) Paraffin, Western Blot (WB)
• Application Notes: IHC-P (7.5 ug/ml); WB (1:500 - 1:3000)
• Immunogen: ATP6V0A4 antibody was raised against recombinant fragment corresponding to a region within amino acids 53 and 295 of V-ATPase 116 kD isoform a4 (SwissProt Q9HBG4). Percent identity by BLAST analysis: Human (100%); Mouse (87%); Bovine (82%).
• Immunogen Description: Recombinant fragment corresponding to a region within amino acids 53 and 295 of V-ATPase 116 kD isoform a4 (SwissProt Q9HBG4). Percent identity by BLAST analysis: Human (100%); Mouse (87%); Bovine (82%).
• Immunogen Type: Recombinant protein
• Antigen Modification: aa53-295
• Target Species: Human
• Preparation and Storage: Keep as concentrated solution. Aliquot and store at -20 degree C or below. Avoid multiple freeze-thaw cycles.

Immunohistochemistry (IHC)

(Anti-ATP6V0A4 antibody IHC staining of human kidney. Immunohistochemistry of formalin-fixed, paraffin-embedded tissue after heat-induced antigen retrieval. Antibody concentration 7.5 ug/ml.)

Western Blot (WB)

(Sample (30 ug of whole cell lysate). A: Hep G2. 7.5% SDS PAGE. ATP6V0A4 antibody diluted at 1:3000.)

Related Product Information for anti-ATP6V0A4 antibody

ATP6V0A4 Antibody, ATP6N1B Antibody, Renal tubular acidosis Antibody, RTA1C Antibody, STV1 Antibody, V-ATPase 116 kDa Antibody, V-ATPase alpha 4 Antibody, RTADR Antibody, ATP6N2 Antibody, RDRTA2 Antibody, V-ATPase 116 kDa isoform a4 Antibody, VPH1 Antibody, VPP2 Antibody Description: ATP6V0A4 is a component of vacuolar ATPase (V-ATPase), a multisubunit enzyme that mediates acidification of intracellular compartments of eukaryotic cells. V-ATPase dependent 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.

NCBI and Uniprot Product Information

• NCBI GI #: 85386547
• NCBI GeneID: 50617
• NCBI Accession #: NP_570856.2
• NCBI GenBank Nucleotide #: NM_130841.2
• UniProt Accession #: Q9HBG4; Q32M47; A4D1R4; A8KA80
• Molecular Weight: 96,386 Da
• NCBI Official Full Name: V-type proton ATPase 116 kDa subunit a isoform 4
• NCBI Official Synonym Full Names: ATPase H+ transporting V0 subunit a4
• NCBI Official Symbol: ATP6V0A4
• NCBI Official Synonym Symbols: A4; STV1; VPH1; VPP2; RTA1C; RTADR; ATP6N2; RDRTA2; ATP6N1B
• NCBI Protein Information: V-type proton ATPase 116 kDa subunit a isoform 4
• UniProt Protein Name: V-type proton ATPase 116 kDa subunit a isoform 4
• Protein Family: V-type proton ATPase
• UniProt Gene Name: ATP6V0A4
• UniProt Synonym Gene Names: ATP6N1B; ATP6N2; V-ATPase 116 kDa isoform a4
• UniProt Entry Name: VPP4_HUMAN

NCBI Description

This gene encodes a component of vacuolar ATPase (V-ATPase), a multisubunit enzyme that mediates acidification of intracellular compartments of eukaryotic cells. V-ATPase dependent 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. This gene is one of four genes in man and mouse that encode different isoforms of the a subunit. Alternatively spliced transcript variants encoding the same protein have been described. Mutations in this gene are associated with renal tubular acidosis associated with preserved hearing. [provided by RefSeq, Jul 2008]

Uniprot Description

ATP6V0A4: Part of the proton channel of the V-ATPase that is involved in normal vectorial acid transport into the urine by the kidney. Defects in ATP6V0A4 are the cause of distal renal tubular acidosis with preserved hearing (RTADR). RTADR is an autosomal recessive form of distal renal tubular acidosis (dRTA), a group of disorders characterized by functional failure of alpha- intercalated cells of the cortical collecting duct of the distal nephron, where vectorial proton transport is required for urinary acidification. Functional failure of alpha-intercalated cells results in metabolic acidosis accompanied by disturbances of potassium balance, urinary calcium solubility, bone physiology and growth. Belongs to the V-ATPase 116 kDa subunit family.

Protein type: Transporter; Transporter, iron; Transporter, ion channel; Energy Metabolism - oxidative phosphorylation; Membrane protein, multi-pass; Membrane protein, integral

Chromosomal Location of Human Ortholog: 7q34

Cellular Component: apical part of cell; apical plasma membrane; brush border membrane; endosome; endosome membrane; integral to membrane; lysosomal membrane; phagocytic vesicle membrane; plasma membrane; vacuolar proton-transporting V-type ATPase complex

Molecular Function: ATPase binding; hydrogen ion transporting ATPase activity, rotational mechanism; protein binding

Biological Process: ATP hydrolysis coupled proton transport; ATP synthesis coupled proton transport; cell redox homeostasis; cellular iron ion homeostasis; excretion; insulin receptor signaling pathway; ossification; proton transport; regulation of pH; sensory perception of sound; transferrin transport; transmembrane transport; vacuolar acidification

Disease: Renal Tubular Acidosis, Distal, Autosomal Recessive