V-Type Proton ATPase 116kDa Subunit A Isoform 4 (ATP6V0A4), ELISA Kit

For Research Use Only. Not for use in diagnostic procedures.

Store all reagents at 2-8 degree C

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Small volumes of ATP6V0A4 elisa kit vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

MBS9908317 is a ready-to-use microwell, strip plate ELISA (enzyme-linked immunosorbent assay) Kit for analyzing the presence of the V-Type Proton ATPase 116kDa Subunit A Isoform 4 (ATP6V0A4) ELISA Kit target analytes in biological samples. The concentration gradients of the kit standards or positive controls render a theoretical kit detection range in biological research samples containing ATP6V0A4. The ELISA analytical biochemical technique of the MBS9908317 kit is based on ATP6V0A4 antibody-ATP6V0A4 antigen interactions (immunosorbency) and an HRP colorimetric detection system to detect ATP6V0A4 antigen targets in samples. The ELISA Kit is designed to detect native, not recombinant, ATP6V0A4. Appropriate sample types may include undiluted body fluids and/or tissue homogenates, secretions. Quality control assays assessing reproducibility identified the intra-assay CV (%) and inter-assay CV(%).

96,386 Da

ATPase H+ transporting V0 subunit a4

V-type proton ATPase 116 kDa subunit a isoform 4; V-type proton ATPase 116 kDa subunit a

V-type proton ATPase 116 kDa subunit a isoform 4

ATP6N1B; ATP6N2; V-ATPase 116 kDa isoform a4  [Similar Products]

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]

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

Chromosomal Location of Human Ortholog: 7q34

Cellular Component: apical part of cell; apical plasma membrane; brush border membrane; endosome; endosome membrane; integral component of 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; excretion; insulin receptor signaling pathway; ossification; proton transport; regulation of pH; sensory perception of sound; transferrin transport; vacuolar acidification

Disease: Renal Tubular Acidosis, Distal, Autosomal Recessive

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