Recombinant人CLCNKB protein (ab117052)

概述

描述

  • 性质Recombinant
  • 来源Wheat germ
  • 氨基酸序列
    • AccessionP51801
    • 种属Human
    • 序列QPSFYDGTVIVKKLPYLPRILGRNIGSHRVRVEHFMNHSITTLAKDTPLE EVVKVVTSTDVAEYPLVESTESQILVGIVRRAQLVQALKAEPPSWAPGHQ
    • 分子量37 kDa including tags
    • 氨基酸516 to 615

技术指标

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.

  • 应用

    Western blot

    SDS-PAGE

    ELISA

  • 形式Liquid
  • 补充说明Protein concentration is above or equal to 0.05 mg/ml.
    Best used within three months from the date of receipt.
  • Concentration information loading...

制备和贮存

  • 稳定性和存储

    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

常规信息

  • 别名
    • 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
  • 功能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.
  • 组织特异性Expressed predominantly in the kidney.
  • 疾病相关Defects 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.
  • 序列相似性Belongs to the chloride channel (TC 2.A.49) family. CLCNKB subfamily.
    Contains 2 CBS domains.
  • 细胞定位Cell membrane.
  • Information by UniProt

Recombinant Human CLCNKB protein 图像

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

Recombinant Human CLCNKB protein (ab117052)参考文献

ab117052 has not yet been referenced specifically in any publications.

Product Wall

There are currently no Abreviews or Questions for ab117052.
Please use the links above to contact us or submit feedback about this product.

Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"