ATP6V0A4 (ATPase, H+ transporting, lysosomal V0 subunit aisoform 4), Polyclonal

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

Polyclonal

IgG

Rabbit

Purified by antigen-specific affinity chromatography.

Liquid

1ug/ul (lot specific)

This product is stable for several weeks at 4 degree C as an undiluted liquid. Dilute only prior to immediate use. For extended storage, aliquot contents and freeze at -20 degree C or below. Avoid cycles of freezing and thawing. Expiration date is one (1) year from date of receipt.

Small volumes of anti-ATP6V0A4 antibody 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.

ATP6V0A4 (ATPase, H+ transporting, lysosomal V0 subunit aisoform 4) is 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, and receptor-mediated endocytosis. V-ATPase is comprised of a cytosolic V1 domain and a transmembrane V0 domain. The V1 domain consists of a hexamer of three A and three B subunits plus the C, D, and E subunits. It contains the ATP catalytic site. The ATP6V0A4 protein is one of three 116 kD subunits family. This family consists of the 116kDa V-type ATPase (vacuolar H+)-ATPases) subunits, as well as V-type ATP synthase subunit i. The V-type ATPases family are proton pumps that acidify intracellular compartments in eukaryotic cells for example yeast central vacuoles, clathrin- coated and synaptic vesicles. They have important roles in membrane trafficking processes. The 116kDa subunit (subunit a) in the V-type ATPase is part of the V0 functional domain responsible for proton transport. The a subunit is a transmembrane glycoprotein with multiple putative transmembrane helices it has a hydrophilic amino terminal and a hydrophobic carboxy terminal. It has roles in proton transport and assembly of the V-type ATPase complex. This subunit is encoded by two homologous gene in yeast VPH1 and STV1

Western Blot (WB), ELISA (EIA)

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 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; 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 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

Smith, et al, Nat. Genet. 26 (1), 71-75 (2000) Karet, F.E., et al, Am. J. Hum. Genet. 65 (6), 1656-1665 (1999) Wieczorek, H., et al, Bioessays 21 (8), 637-648 (1999) Forgac, M., J. Biol. Chem. 274 (19), 12951-12954 (1999) Nelson, N. and Harvey, W.R., Physiol. Rev. 79 (2), 361-385 (1999)

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