Western Blot (WB)
(ATP6V0A4 Antibody (Center) western blot analysis in CEM cell line lysates (35ug/lane).This demonstrates the ATP6V0A4 antibody detected the ATP6V0A4 protein (arrow).)
Rabbit anti-Human ATP6V0A4 Polyclonal Antibody | anti-ATP6V0A4 antibody
ATP6V0A4 Antibody (Center)
Western Blot (WB)
(ATP6V0A4 Antibody (Center) western blot analysis in CEM cell line lysates (35ug/lane).This demonstrates the ATP6V0A4 antibody detected the ATP6V0A4 protein (arrow).)
Western Blot (WB)
(ATP6V0A4 Antibody (Center) western blot analysis in CEM cell line lysates (35ug/lane).This demonstrates the ATP6V0A4 antibody detected the ATP6V0A4 protein (arrow).)
Flow Cytometry (FC/FACS)
(ATP6V0A4 Antibody (Center) flow cytometric analysis of CEM cells (right histogram) compared to a negative control cell (left histogram).FITC-conjugated goat-anti-rabbit secondary antibodies were used for the analysis.)
Flow Cytometry (FC/FACS)
(ATP6V0A4 Antibody (Center) flow cytometric analysis of CEM cells (right histogram) compared to a negative control cell (left histogram).FITC-conjugated goat-anti-rabbit secondary antibodies were used for the analysis.)
(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.
Hinton, A., et al. J. Biol. Chem. 284(24):16400-16408(2009)
Su, Y., et al. Am. J. Physiol. Renal Physiol. 295 (4), F950-F958 (2008) :
Norgett, E.E., et al. J. Biol. Chem. 282(19):14421-14427(2007)
Smith, A.N., et al. J. Am. Soc. Nephrol. 16(5):1245-1256(2005)
Forgac, M. J. Biol. Chem. 274(19):12951-12954(1999)
Nelson, N., et al. Physiol. Rev. 79(2):361-385(1999)
Kane, P.M. J. Bioenerg. Biomembr. 31(1):3-5(1999)
Finbow, M.E., et al. Biochem. J. 324 (PT 3), 697-712 (1997) :
Stevens, T.H., et al. Annu. Rev. Cell Dev. Biol. 13, 779-808 (1997) :
NCBI and Uniprot Product Information
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: Energy Metabolism - oxidative phosphorylation; Membrane protein, multi-pass; Membrane protein, integral; Transporter, iron; Transporter, ion channel; Transporter
Chromosomal Location of Human Ortholog: 7q34
Cellular Component: phagocytic vesicle membrane; brush border membrane; apical part of cell; lysosomal membrane; apical plasma membrane; plasma membrane; integral to 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: interaction with host; ossification; ATP synthesis coupled proton transport; cellular iron ion homeostasis; transferrin transport; excretion; vacuolar acidification; proton transport; sensory perception of sound; ATP hydrolysis coupled proton transport; insulin receptor signaling pathway; regulation of pH; transmembrane transport
Disease: Renal Tubular Acidosis, Distal, Autosomal Recessive
Research Articles on ATP6V0A4
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Product Notes
The ATP6V0A4 atp6v0a4 (Catalog #AAA9214835) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The immunogen sequence is 235-261. The ATP6V0A4 Antibody (Center) reacts with Human and may cross-react with other species as described in the data sheet. AAA Biotech's ATP6V0A4 can be used in a range of immunoassay formats including, but not limited to, Western Blot (WB), ELISA (EIA), Flow Cytometry (FC/FACS). WB~~1:1000. Researchers should empirically determine the suitability of the ATP6V0A4 atp6v0a4 for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "ATP6V0A4, Polyclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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