Recombinant Human CLCNKB protein (ab117052)

Overview

Description

  • NatureRecombinant
  • SourceWheat germ
  • Amino Acid Sequence
    • AccessionP51801
    • SpeciesHuman
    • SequenceQPSFYDGTVIVKKLPYLPRILGRNIGSHRVRVEHFMNHSITTLAKDTPLE EVVKVVTSTDVAEYPLVESTESQILVGIVRRAQLVQALKAEPPSWAPGHQ
    • Molecular weight37 kDa including tags
    • Amino acids516 to 615

Specifications

Our Abpromise guarantee covers the use of ab117052 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

  • Applications

    Western blot

    SDS-PAGE

    ELISA

  • FormLiquid
  • Additional notesProtein concentration is above or equal to 0.05 mg/ml.
    Best used within three months from the date of receipt.
  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.

    pH: 8.00
    Constituents: 0.3% Glutathione, 0.79% Tris HCl

General Info

  • Alternative names
    • Bartter syndrome type 3
    • Chloride channel Kb
    • Chloride channel kidney B
    • Chloride channel protein ClC-Kb
    • Chloride channel voltage sensitive Kb
    • ClC K2
    • ClC-K2
    • ClCK2
    • CLCKB
    • CLCKB_HUMAN
    • CLCNKB
    • hClC Kb
    • hClCKb
    • MGC24087
    • OTTHUMP00000011120
    • OTTHUMP00000011121
    • RP11 5P18.8
    see all
  • FunctionVoltage-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.
  • Tissue specificityExpressed predominantly in the kidney.
  • Involvement in diseaseDefects in CLCNKB are the cause of Bartter syndrome type 3 (BS3) [MIM:607364]; 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) [MIM:613090]. 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.
  • Sequence similaritiesBelongs to the chloride channel (TC 2.A.49) family. CLCNKB subfamily.
    Contains 2 CBS domains.
  • Cellular localizationCell membrane.
  • Information by UniProt

Recombinant Human CLCNKB protein images

  • 12.5% SDS-PAGE showing ab117052 at approximately 36.63 kDa.
    Stained with Coomassie Blue.

References for Recombinant Human CLCNKB protein (ab117052)

ab117052 has not yet been referenced specifically in any publications.

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