Barttin; BSND; BART

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

Polyclonal

Rabbit Ig

Rabbit

This BSND antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 292-320 amino acids from the C-terminal region of human BSND.

Peptide Affinity Purified Rabbit Polyclonal Antibody (Pab)

Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification.

Vial Concentration: 0.5 (lot specific)

Maintain refrigerated at 2-8 degree C for up to 6 months. For long term storage store at -20 degree C in small aliquots to prevent freeze-thaw cycles.

Small volumes of anti-BSND 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.

BSND encodes an essential beta subunit for CLC chloride channels. These heteromeric channels localize to basolateral membranes of renal tubules and of potassium-secreting epithelia of the inner ear.

Neuroscience; Signal Transduction

Western Blot (WB), ELISA (EIA), Immunohistochemistry (IHC)

WB~~1:1000

Western blot analysis of BSND Antibody (C-term) in mouse liver tissue lysates (35ug/lane). BSND (arrow) was detected using the purified Pab.

BSND Antibody (C-term) IHC analysis in formalin fixed and paraffin embedded mouse kidney tissue followed by peroxidase conjugation of the secondary antibody and DAB staining. This data demonstrates the use of the BSND Antibody (C-term) for immunohistochemistry. Clinical relevance has not been evaluated.

35197

barttin CLCNK-type chloride channel accessory beta subunit

barttin

Barttin

BART??[Similar Products]

BSND_HUMAN

This gene encodes an essential beta subunit for CLC chloride channels. These heteromeric channels localize to basolateral membranes of renal tubules and of potassium-secreting epithelia of the inner ear. Mutations in this gene have been associated with Bartter syndrome with sensorineural deafness. [provided by RefSeq, Jul 2008]

BSND: Functions as a beta-subunit for CLCNKA and CLCNKB chloride channels. In the kidney CLCNK/BSND heteromers mediate chloride reabsorption by facilitating its basolateral efflux. In the stria, CLCNK/BSND channels drive potassium secretion by recycling chloride for the basolateral SLC12A2 cotransporter. Defects in BSND are the cause of Bartter syndrome type 4A (BS4A); also known as infantile Bartter syndrome with sensorineural deafness. BS refers to a group of autosomal recessive disorders characterized by impaired salt reabsorption in the thick ascending loop of Henle with pronounced salt wasting, hypokalemic metabolic alkalosis, and varying degrees of hypercalciuria. BS4A is associated with sensorineural deafness.

Protein type: Transporter; Membrane protein, multi-pass; Transporter, ion channel; Membrane protein, integral

Chromosomal Location of Human Ortholog: 1p32.1

Cellular Component: protein complex; integral to plasma membrane; basolateral plasma membrane; cytoplasm; plasma membrane

Molecular Function: chloride channel activity; chloride channel regulator activity

Biological Process: transmembrane transport

Disease: Bartter Syndrome, Type 4a

Riazuddin, S., et al. Am. J. Hum. Genet. 85(2):273-280(2009)
Brochard, K., et al. Nephrol. Dial. Transplant. 24(5):1455-1464(2009)
Bircan, Z., et al. Pediatr. Nephrol. 24(4):841-844(2009)
Kathiresan, S., et al. Nat. Genet. 41(3):334-341(2009)
Kathiresan, S., et al. Nat. Genet. 41(1):56-65(2009)

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