Western Blot (WB)
(ATP6V1B1 Antibody (Center) western blot analysis in WiDr,K562 cell line lysates (35ug/lane).This demonstrates the ATP6V1B1 antibody detected the ATP6V1B1 protein (arrow).)
Rabbit ATP6V1B1 Polyclonal Antibody | anti-ATP6V1B1 antibody
ATP6V1B1 Antibody (Center)
Western Blot (WB)
(ATP6V1B1 Antibody (Center) western blot analysis in WiDr,K562 cell line lysates (35ug/lane).This demonstrates the ATP6V1B1 antibody detected the ATP6V1B1 protein (arrow).)
Western Blot (WB)
(ATP6V1B1 Antibody (Center) western blot analysis in WiDr,K562 cell line lysates (35ug/lane).This demonstrates the ATP6V1B1 antibody detected the ATP6V1B1 protein (arrow).)
Immunohistochemistry (IHC)
(ATP6V1B1 Antibody (Center) immunohistochemistry analysis in formalin fixed and paraffin embedded human kidney tissue followed by peroxidase conjugation of the secondary antibody and DAB staining.This data demonstrates the use of ATP6V1B1 Antibody (Center) for immunohistochemistry. Clinical relevance has not been evaluated.)
Immunohistochemistry (IHC)
(ATP6V1B1 Antibody (Center) immunohistochemistry analysis in formalin fixed and paraffin embedded human kidney tissue followed by peroxidase conjugation of the secondary antibody and DAB staining.This data demonstrates the use of ATP6V1B1 Antibody (Center) for immunohistochemistry. Clinical relevance has not been evaluated.)
Immunofluorescence (IF)
(Confocal immunofluorescent analysis of ATP6V1B1 Antibody (Center) with WiDr cell followed by Alexa Fluor 488-conjugated goat anti-rabbit lgG (green). DAPI was used to stain the cell nuclear (blue).)
Immunofluorescence (IF)
(Confocal immunofluorescent analysis of ATP6V1B1 Antibody (Center) with WiDr cell followed by Alexa Fluor 488-conjugated goat anti-rabbit lgG (green). DAPI was used to stain the cell nuclear (blue).)
(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, 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. Additional isoforms of
many of the V1 and V0 subunit proteins are encoded by multiple
genes or alternatively spliced transcript variants. This encoded
protein is one of two V1 domain B subunit isoforms and is found in
the kidney. Mutations in this gene cause distal renal tubular
acidosis associated with sensorineural deafness. [provided by
RefSeq].
Sharifian, M., et al. Iran J Kidney Dis 4(3):202-206(2010)
Talmud, P.J., et al. Am. J. Hum. Genet. 85(5):628-642(2009)
Andreucci, E., et al. Pediatr. Nephrol. 24(11):2147-2153(2009)
Sethi, S.K., et al. Indian Pediatr 46(5):425-427(2009)
NCBI and Uniprot Product Information
NCBI Description
This gene encodes 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, 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. Additional isoforms of many of the V1 and V0 subunit proteins are encoded by multiple genes or alternatively spliced transcript variants. This encoded protein is one of two V1 domain B subunit isoforms and is found in the kidney. Mutations in this gene cause distal renal tubular acidosis associated with sensorineural deafness. [provided by RefSeq, Jul 2008]
Uniprot Description
ATP6V1B1: Non-catalytic subunit of the peripheral V1 complex of vacuolar ATPase. V-ATPase is responsible for acidifying a variety of intracellular compartments in eukaryotic cells. Defects in ATP6V1B1 are the cause of distal renal tubular acidosis with deafness (dRTA-D). Inheritance is autosomal recessive. Patients with recessive dRTA are severely affected, presenting with either acute illness or growth failure at a young age, and bilateral sensorineural deafness. Other features include low serum K(+) due to renal potassium wasting, and elevated urinary calcium. If untreated, this acidosis may result in dissolution of bone, leading to osteomalacia and rickets. Renal deposition of calcium salts (nephrocalcinosis) and renal stone formation commonly occur. Belongs to the ATPase alpha/beta chains family.
Protein type: Energy Metabolism - oxidative phosphorylation; EC 3.6.3.14; Hydrolase
Chromosomal Location of Human Ortholog: 2p13.1
Cellular Component: microvillus; basolateral plasma membrane; cytoplasm; apical plasma membrane; endomembrane system; vacuolar proton-transporting V-type ATPase complex; cytosol; lateral plasma membrane
Molecular Function: protein complex binding; hydrogen ion transmembrane transporter activity; ATP binding; hydrolase activity, acting on acid anhydrides, catalyzing transmembrane movement of substances
Biological Process: interaction with host; inner ear morphogenesis; ossification; cellular iron ion homeostasis; transferrin transport; excretion; ATP metabolic process; calcium ion homeostasis; proton transport; sensory perception of sound; ATP hydrolysis coupled proton transport; pH reduction; insulin receptor signaling pathway; regulation of pH; transmembrane transport
Disease: Renal Tubular Acidosis, Distal, With Progressive Nerve Deafness
Research Articles on ATP6V1B1
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Product Notes
The ATP6V1B1 atp6v1b1 (Catalog #AAA9201826) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The immunogen sequence is 284-310. The ATP6V1B1 Antibody (Center) reacts with Human (Predicted Reactivity: Bovine, Mouse, Rat, C Elegans, Chicken, Drosophila) and may cross-react with other species as described in the data sheet. AAA Biotech's ATP6V1B1 can be used in a range of immunoassay formats including, but not limited to, Western Blot (WB), ELISA (EIA), Immunohistochemistry (IHC), Immunofluorescence (IF). WB~~1:1000. Researchers should empirically determine the suitability of the ATP6V1B1 atp6v1b1 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 "ATP6V1B1, 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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