Product Name
CLCNKB, Polyclonal Antibody
Full Product Name
CLCNKB Antibody
Product Synonym Names
CLCNKB; Bartter syndrome Type 3; Chloride channel; kidney; B; Chloride channel Kb; ClC-Kb; ClC-K2; CLCKB; HClC-Kb
Product Gene Name
anti-CLCNKB antibody
[Similar Products]
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
3D Structure
ModBase 3D Structure for P51801
Specificity
CLCNKB Antibody detects endogenous levels of total CLCNKB
Form/Format
Rabbit IgG in phosphate buffered saline, pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.
Concentration
1.0mg/mL (lot specific)
Immunogen
A synthesized peptide derived from human CLCNKB
Preparation and Storage
Store at -20 degree C
Other Notes
Small volumes of anti-CLCNKB 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.
Product Categories/Family for anti-CLCNKB antibody
Total protein Ab
Applications Tested/Suitable for anti-CLCNKB antibody
Western Blot (WB)
Application Notes for anti-CLCNKB antibody
Western Blot: 1:1000-3000
Western Blot (WB) of anti-CLCNKB antibody
Western blot analysis CLCNKB using COLO205 whole cell lysates
NCBI/Uniprot data below describe general gene information for CLCNKB. It may not necessarily be applicable to this product.
NCBI Accession #
NP_000076.2
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NCBI GenBank Nucleotide #
NM_000085.4
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UniProt Primary Accession #
P51801
[Other Products]
UniProt Secondary Accession #
Q5T5Q7; Q5T5Q8; B3KUY3[Other Products]
UniProt Related Accession #
P51801[Other Products]
Molecular Weight
56,998 Da
NCBI Official Full Name
chloride channel protein ClC-Kb isoform 1
NCBI Official Synonym Full Names
chloride voltage-gated channel Kb
NCBI Official Symbol
CLCNKB [Similar Products]
NCBI Official Synonym Symbols
CLCKB; ClC-K2; ClC-Kb
[Similar Products]
NCBI Protein Information
chloride channel protein ClC-Kb
UniProt Protein Name
Chloride channel protein ClC-Kb
UniProt Synonym Protein Names
ClC-K2
Protein Family
Chloride channel protein
UniProt Gene Name
CLCNKB [Similar Products]
UniProt Synonym Gene Names
Chloride channel Kb [Similar Products]
UniProt Entry Name
CLCKB_HUMAN
NCBI Summary for CLCNKB
The protein encoded by this gene is a member of the family of voltage-gated chloride channels. Chloride channels have several functions, including the regulation of cell volume, membrane potential stabilization, signal transduction and transepithelial transport. This gene is expressed predominantly in the kidney and may be important for renal salt reabsorption. Mutations in this gene are associated with autosomal recessive Bartter syndrome type 3 (BS3). Alternatively spliced transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Sep 2009]
UniProt Comments for CLCNKB
CLCNKB: Voltage-gated chloride channel. Chloride channels have several functions including the regulation of cell volume; membrane potential stabilization, signal transduction and transepithelial transport. May be important in urinary concentrating mechanisms. Defects in CLCNKB are the cause of Bartter syndrome type 3 (BS3); also known as classic Bartter syndrome. It is an autosomal recessive form of often severe intravascular volume depletion due to renal salt-wasting associated with low blood pressure, hypokalemic alkalosis, hypercalciuria, and normal serum magnesium levels. Defects in CLCNKB are a cause of Bartter syndrome type 4B (BS4B). A digenic, recessive disorder characterized by impaired salt reabsorption in the thick ascending loop of Henle with pronounced salt wasting, hypokalemic metabolic alkalosis, and varying degrees of hypercalciuria. Bartter syndrome type 4B is associated with sensorineural deafness. Belongs to the chloride channel (TC 2.A.49) family. CLCNKB subfamily.
Protein type: Membrane protein, integral; Membrane protein, multi-pass; Transporter; Transporter, ion channel
Chromosomal Location of Human Ortholog: 1p36
Cellular Component: integral to plasma membrane; plasma membrane
Molecular Function: chloride ion binding; voltage-gated chloride channel activity
Biological Process: excretion; transport
Disease: Bartter Syndrome, Type 3; Bartter Syndrome, Type 4b
Research Articles on CLCNKB
1. Five patients had 1 or more mutations in CLCNKB, of whom 3 had homozygous mutations and 2 had single heterozygous mutations and only in CLCNKB had hypocalciuria.
Precautions
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Disclaimer
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