• Call +1.858.633.0165 or Fax +1.858.633.0166 or Contact Us

BSND recombinant protein :: Bartter Syndrome Infantile with Sensorineural Deafness Recombinant Protein

Scan QR to view Datasheet
Catalog # MBS146293
Unit / Price
  0.002 mg  /  $140 +1 FREE 8GB USB
  0.01 mg  /  $205 +1 FREE 8GB USB
  0.1 mg  /  $1,220 +1 FREE 8GB USB
BSND recombinant protein
Product Name

Bartter Syndrome Infantile with Sensorineural Deafness (BSND), Recombinant Protein

Also Known As

Recombinant Human Bartter Syndrome Infantile with Sensorineural Deafness

Product Synonym Names
BSND Human; Bartter Syndrome Infantile with Sensorineural Deafness Human Recombinant; Bartter Syndrome Infantile With Sensorineural Deafness (Barttin); Deafness Autosomal Recessive 73; DFNB73; BART; barttin
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
Sequence Length
320
Sequence
MGSSHHH HHH SSGLVPRGSH MGSCQC YPKI TFVPADSDFQ GILSPKAMGL LENGLAAEMK SPSPQPPYVR LWEEAAYDQS LPDFSHIQMK VMSYSEDHRS LLAPEMGQPK LGTSDGGEGG PGDVQAWMEA AVVIHKGSDE SEGERRLTQS WPGPLACPQG PAPLASFQDD LDMDSSEGSS PNASPHDREE ACSPQQEPQG CRCPLDRFQD FALIDAPTLE DEPQEGQQWE IALPNNWQRY PRTKVEEKEA SDTGGEEPEK EEEDLYYGLP DGAGDLLPDK ELGFEPDTQG
OMIM
602522
3D Structure
ModBase 3D Structure for Q8WZ55
Host
E Coli
Purity/Purification
Greater than 90% as determined by SDS-PAGE.
Form/Format
The BSND solution (0.25mg/ml) contains 20mM Tris-HCl buffer (pH 8.0), 0.15M NaCl, 1mM DTT and 10% glycerol.
Sterile Filtered clear solution.
Other Notes
Small volumes of BSND recombinant protein 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
BSND recombinant protein
Description: BSND Human Recombinant produced in E Coli is a single, non-glycosylated polypeptide chain containing 290 amino acids (54-320) and having a molecular mass of 31.7kDa.BSND is fused to a 23 amino acid His-tag at N-terminus & purified by proprietary chromatographic techniques.

Introduction: BSND is a vital beta subunit for CLC chloride channels. These heteromeric channels are restricted to basolateral membranes of renal tubules and of potassium-secreting epithelia of the inner ear. BSND gene mutations are linked with Bartter syndrome with sensorineural deafness.
NCBI/Uniprot data below describe general gene information for BSND. It may not necessarily be applicable to this product.
NCBI GI #
NCBI GeneID
NCBI Accession #
NCBI GenBank Nucleotide #
UniProt Primary Accession #
UniProt Secondary Accession #
UniProt Related Accession #
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
NCBI Official Synonym Symbols
BART; DFNB73
  [Similar Products]
NCBI Protein Information
barttin; Bartter syndrome, infantile, with sensorineural deafness (Barttin); deafness, autosomal recessive 73
UniProt Protein Name
Barttin
Protein Family
UniProt Gene Name
UniProt Synonym Gene Names
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, 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
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.
Organs/Tissues associated with BSND recombinant protein
Request a Quote

Please fill out the form below and our representative will get back to you shortly.

MBS000000
Contact Us

Please fill out the form below and our representative will get back to you shortly.

MBS000000