Western Blot (WB)
(human kidney tissue were subjected to SDS PAGE followed by western blot with MBS766949 (ATP6V1B1 antibody) at dilution of 1:1000)
Rabbit ATP6V1B1 Polyclonal Antibody | anti-ATP6V1B1 antibody
ATP6V1B1 Rabbit Polyclonal
Immunogen Affinity Purified
Immunogen Affinity Purified
Western Blot (WB)
(human kidney tissue were subjected to SDS PAGE followed by western blot with MBS766949 (ATP6V1B1 antibody) at dilution of 1:1000)
Western Blot (WB)
(human kidney tissue were subjected to SDS PAGE followed by western blot with MBS766949 (ATP6V1B1 antibody) at dilution of 1:1000)
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: Hydrolase; Energy Metabolism - oxidative phosphorylation; EC 3.6.3.14
Chromosomal Location of Human Ortholog: 2p13.1
Cellular Component: apical plasma membrane; basolateral plasma membrane; cytoplasm; cytosol; endomembrane system; lateral plasma membrane; microvillus; vacuolar proton-transporting V-type ATPase complex
Molecular Function: ATP binding; hydrogen ion transmembrane transporter activity; hydrolase activity, acting on acid anhydrides, catalyzing transmembrane movement of substances; protein complex binding
Biological Process: ATP hydrolysis coupled proton transport; ATP metabolic process; calcium ion homeostasis; excretion; inner ear morphogenesis; insulin receptor signaling pathway; ossification; pH reduction; proton transport; regulation of macroautophagy; regulation of pH; sensory perception of sound; transferrin transport
Disease: Renal Tubular Acidosis, Distal, With Progressive Nerve Deafness
Research Articles on ATP6V1B1
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Product Notes
The ATP6V1B1 atp6v1b1 (Catalog #AAA766949) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The ATP6V1B1 Rabbit Polyclonal reacts with Human, Mouse, Rat 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, ELISA (EIA), Western Blot (WB). 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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