Rabbit anti-Human, Mouse BSND Polyclonal Antibody | anti-BSND antibody

BSND, CT (BSND, BART, Barttin) (FITC)

Cat. Num. AAA6279080

• Gene Names: BSND; BART; DFNB73
• Reactivity: Human, Mouse
• Applications: Immunohistochemistry, Western Blot
• Purity: Purified by Protein A Affinity Chromatography.
• Synonyms: BSND; Polyclonal Antibody; CT (BSND; BART; Barttin) (FITC); Barttin; anti-BSND antibody

• Host: Rabbit
• Reactivity: Human, Mouse
• Clonality: Polyclonal
• Isotype: IgG
• Purity/Purification: Purified by Protein A Affinity Chromatography.
• Form/Format: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Fluorescein Isothiocyanate (FITC).

• Applicable Applications for anti-BSND antibody: Immunohistochemistry (IHC), FLISA, Western Blot (WB)
• Application Notes: IHC: 1:50-100; FLISA: 1:1,000; WB: 1:100-500; Applications are based on unconjugated antibody.
• Immunogen: BSND antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 291~320 amino acids from the C-terminal region of human BSND.
• Conjugate: FITC
• Note: Preservative Free
• Special Handling: Light sensitive
• Preparation and Storage: Store product at 4 degree C if to be used immediately within two weeks. For long-term storage, aliquot to avoid repeated freezing and thawing and store at -20 degree C. Aliquots are stable at -20 degree C for 12 months after receipt. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. Caution: FITC conjugates are sensitive to light. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Western Blot (WB)

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

Immunohistochemistry (IHC)

(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.)

Related Product Information for anti-BSND antibody

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.

Product Categories/Family for anti-BSND antibody

Antibodies; Proteins

References

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).

NCBI and Uniprot Product Information

• NCBI GI #: 17017967
• NCBI GeneID: 7809
• NCBI Accession #: NP_476517.1
• NCBI GenBank Nucleotide #: NM_057176.2
• UniProt Accession #: Q8WZ55; Q6NT28
• Molecular Weight: 35,197 Da
• NCBI Official Full Name: barttin
• NCBI Official Synonym Full Names: barttin CLCNK-type chloride channel accessory beta subunit
• NCBI Official Symbol: BSND
• NCBI Official Synonym Symbols: BART; DFNB73
• NCBI Protein Information: barttin; Bartter syndrome, infantile, with sensorineural deafness (Barttin); deafness, autosomal recessive 73
• UniProt Protein Name: Barttin
• Protein Family: Barttin
• UniProt Gene Name: BSND
• UniProt Synonym Gene Names: BART
• UniProt Entry Name: BSND_HUMAN

NCBI Description

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]

Uniprot Description

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

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