Human ATPase, H+ transporting, lysosomal V0 subunit a4 ELISA Kit | ATP6V0A4 elisa kit

Human V-type proton ATPase 116 kDa subunit a isoform 4, ATP6V0A4 ELISA Kit

Cat. Num. AAA9327390

• Gene Names: ATP6V0A4; A4; STV1; VPH1; VPP2; RTA1C; RTADR; ATP6N2; RDRTA2; ATP6N1B
• Reactivity: Human
• Synonyms: ATPase; H+ transporting; lysosomal V0 subunit a4; Human V-type proton ATPase 116 kDa subunit a isoform 4; ATP6V0A4 ELISA Kit; Human V-type proton ATPase 116 kDa subunit a isoform 4 (ATP6V0A4) ELISA kit; tcag7.346; A4; ATP6N1B; ATP6N2; MGC130016; MGC130017; RDRTA2; RTA1C; RTADR; STV1; VPH1; VPP2; lysosomal (vacuolar proton pump) non-catalytic accessory protein 1B; H+; ATP6V0A4 elisa kit

• Reactivity: Human
• Specificity: No significant cross-reactivity or interference between this analyte and analogues is observed.

• Samples: Body fluids, tissue homogenates, secretions or feces samples
• Assay Type: Quantitative Sandwich
• Detection Range: 0.625 ng/ml - 20 ng/ml
• Sensitivity: 0.1 ng/ml.
• Intra-assay Precision: Intra-assay CV (%) is less than 15%
• Inter-assay Precision: Inter-assay CV (%) is less than 15%. [CV(%) = SD/mean ×100].
• Preparation and Storage: Store all reagents at 2-8 degree C

Related Product Information for ATP6V0A4 elisa kit

Background/Introduction: This Quantitative Sandwich ELISA kit is only for in vitro research use only! This kit is intended to be used for determination the level of ATP6V0A4 (hereafter termed "analyte") in undiluted original Human body fluids, tissue homogenates, secretions or feces samples. This kit is NOT suitable for assaying non-biological sources of substances.

NCBI and Uniprot Product Information

• NCBI GI #: 85386053
• NCBI GeneID: 50617
• NCBI Accession #: NP_065683.2
• NCBI GenBank Nucleotide #: NM_020632.2
• 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, lysosomal 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; V-ATPase 116 kDa; vacuolar proton pump, subunit 2; vacuolar proton pump 116 kDa accessory subunit; vacuolar proton translocating ATPase 116 kDa subunit a kidney isoform; H(+)-transporting two-sector ATPase, noncatalytic accessory protein 1B; ATPase, H+ transporting, lysosomal (vacuolar proton pump) non-catalytic accessory protein 1B; ATPase, H+ transporting, lysosomal (vacuolar proton pump) non-catalytic accessory protein 2 (38kD)
• 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, ion channel; Membrane protein, integral; Transporter; Energy Metabolism - oxidative phosphorylation; Membrane protein, multi-pass; Transporter, iron

Chromosomal Location of Human Ortholog: 7q34

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

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

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

Disease: Renal Tubular Acidosis, Distal, Autosomal Recessive