Full Product Name
BSND (Barttin, Bartter Syndrome, Infantile, with Sensorineural Deafness, BART, DFNB73, MGC119283, MGC119284, MGC119285)
Product Synonym Names
Anti -BSND (Barttin, Bartter Syndrome, Infantile, with Sensorineural Deafness, BART, DFNB73, MGC119283, MGC119284, MGC119285)
Product Gene Name
anti-BSND antibody
[Similar Products]
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
Immunogen Sequence
ACSPQQEPQG CRCPLDRFQD FALIDAPTLE DEPQEGQQWE IALPNNWQRY PRTKVEEKEA SDTGGEEPEK EEEDLYYGLP DGAGDLLPDK ELGFEPDTQG
Chromosome Location
Chromosome: 1; NC_000001.10 (55464617..55474465). Location: 1p32.1
3D Structure
ModBase 3D Structure for Q8WZ55
Specificity
Recognizes human BSND.
Purity/Purification
Affinity Purified
Purified by Protein A affinity chromatography.
Form/Format
Supplied as a liquid in PBS, pH 7.2.
Immunogen
Partial recombinant corresponding to aa221-321 from human BSND (NP_476517) with GST tag. MW of the GST tag alone is 26kD.
Preparation and Storage
May be stored at 4 degree C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degree C. Aliquots are stable for 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
Other Notes
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.
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; Abs to Disease Markers
Applications Tested/Suitable for anti-BSND antibody
ELISA (EL/EIA), Western Blot (WB)
Application Notes for anti-BSND antibody
Suitable for use in ELISA and Western Blot.
NCBI/Uniprot data below describe general gene information for BSND. It may not necessarily be applicable to this product.
NCBI Accession #
NP_476517.1
[Other Products]
NCBI GenBank Nucleotide #
NM_057176.2
[Other Products]
UniProt Primary Accession #
Q8WZ55
[Other Products]
UniProt Secondary Accession #
Q6NT28[Other Products]
UniProt Related Accession #
Q8WZ55[Other Products]
Molecular Weight
35,197 Da[Similar Products]
NCBI Official Full Name
barttin
NCBI Official Synonym Full Names
Bartter syndrome, infantile, with sensorineural deafness (Barttin)
NCBI Official Symbol
BSND??[Similar Products]
NCBI Official Synonym Symbols
BART; DFNB73
??[Similar Products]
NCBI Protein Information
barttin; deafness, autosomal recessive 73
UniProt Protein Name
Barttin
UniProt Gene Name
BSND??[Similar Products]
UniProt Synonym Gene Names
BART??[Similar Products]
UniProt Entry Name
BSND_HUMAN
NCBI Summary for BSND
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 Comments for BSND
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, multi-pass; Transporter, ion channel; Membrane protein, integral; Transporter
Chromosomal Location of Human Ortholog: 1p32.1
Cellular Component: protein complex; basolateral plasma membrane; integral to plasma membrane; cytoplasm; plasma membrane
Molecular Function: chloride channel activity; chloride channel regulator activity
Biological Process: transmembrane transport
Disease: Bartter Syndrome, Type 4a
Research Articles on BSND
1. BSND, was first modeled, and then, the identified mutation was further analyzed by using different bioinformatics tools.
Precautions
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.
Disclaimer
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.
