ATP6V1B1, Polyclonal Antibody

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

Polyclonal

Rabbit

Small volumes of anti-ATP6V1B1 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.

Polyclonal

Western Blot (WB), Immunohistochemistry (IHC)

Western blot analysis of extracts of various cell lines, using ATP6V1B1 antibody at 1:1000 dilution.
Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (MBS128200) at 1:10000 dilution.
Lysates/proteins: 25ug per lane.
Blocking buffer: 3% nonfat dry milk in TBST.
Detection: ECL Basic Kit.
Exposure time: 90s.

513

ATPase, H+ transporting, lysosomal 56/58kDa, V1 subunit B1

V-type proton ATPase subunit B, kidney isoform; V-ATPase B1 subunit; V-ATPase subunit B 1; vacuolar proton pump 3; H+-ATPase beta 1 subunit; vacuolar proton pump, subunit 3; vacuolar proton pump subunit B 1; endomembrane proton pump 58 kDa subunit; H(+)-transporting two-sector ATPase, 58kD subunit

V-type proton ATPase subunit B, kidney isoform

ATP6B1; VATB; VPP3; V-ATPase subunit B 1  [Similar Products]

VATB1_HUMAN

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]

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; Hydrolase; EC 3.6.3.14

Chromosomal Location of Human Ortholog: 2p13.1

Cellular Component: microvillus; basolateral plasma membrane; apical plasma membrane; cytoplasm; endomembrane system; vacuolar proton-transporting V-type ATPase complex; cytosol; lateral plasma membrane

Molecular Function: protein complex binding; hydrogen ion transmembrane transporter activity; hydrolase activity, acting on acid anhydrides, catalyzing transmembrane movement of substances; ATP binding

Biological Process: interaction with host; ossification; inner ear morphogenesis; cellular iron ion homeostasis; transferrin transport; excretion; calcium ion homeostasis; ATP metabolic process; 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

All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.

While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.