Recombinant人CFTR protein (ab114246)

概述

  • 产品名称Recombinant人CFTR protein
  • 蛋白长度Protein fragment

描述

  • 性质Recombinant
  • 来源Wheat germ
  • 氨基酸序列
    • AccessionP13569
    • 种属Human
    • 序列YQIIRRTLKQAFADCTVILCEHRIEAMLECQQFLVIEENKVRQYDSIQKL LNERSLFRQAISPSDRVKLFPHRNSSKCKSKPQIAALKEETEEEVQDTRL
    • 分子量37 kDa including tags
    • 氨基酸1381 to 1480

技术指标

Our Abpromise guarantee covers the use of ab114246 in the following tested applications.

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

  • 应用

    ELISA

    SDS-PAGE

    Western blot

  • 形式Liquid
  • 补充说明Protein concentration is above or equal to 0.05 µg/µl
  • 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
    • ABC 35
    • ABC35
    • ABCC 7
    • ABCC7
    • ATP binding cassette sub family C member 7
    • ATP Binding Cassette Superfamily C Member 7
    • ATP binding cassette transporter sub family C member 7
    • ATP-binding cassette sub-family C member 7
    • cAMP dependent chloride channel
    • cAMP-dependent chloride channel
    • CF
    • CFTR
    • CFTR/MRP
    • CFTR_HUMAN
    • Channel conductance controlling ATPase
    • Channel conductance-controlling ATPase
    • Cystic fibrosis transmembrane conductance regulator
    • Cystic fibrosis transmembrane conductance regulator ATP binding cassette sub family C member 7
    • Cystic Fibrosis Transmembrane Regulator
    • dJ760C5.1
    • MRP 7
    • MRP7
    • TNR CFTR
    see all
  • 功能Involved in the transport of chloride ions. May regulate bicarbonate secretion and salvage in epithelial cells by regulating the SLC4A7 transporter.
  • 组织特异性Found on the surface of the epithelial cells that line the lungs and other organs.
  • 疾病相关Defects in CFTR are the cause of cystic fibrosis (CF) [MIM:219700]; also known as mucoviscidosis. CF is the most common genetic disease in the Caucasian population, with a prevalence of about 1 in 2'000 live births. Inheritance is autosomal recessive. CF is a common generalized disorder of exocrine gland function which impairs clearance of secretions in a variety of organs. It is characterized by the triad of chronic bronchopulmonary disease (with recurrent respiratory infections), pancreatic insufficiency (which leads to malabsorption and growth retardation) and elevated sweat electrolytes.
    Defects in CFTR are the cause of congenital bilateral absence of the vas deferens (CBAVD) [MIM:277180]. CBAVD is an important cause of sterility in men and could represent an incomplete form of cystic fibrosis, as the majority of men suffering from cystic fibrosis lack the vas deferens.
  • 序列相似性Belongs to the ABC transporter superfamily. ABCC family. CFTR transporter (TC 3.A.1.202) subfamily.
    Contains 2 ABC transmembrane type-1 domains.
    Contains 2 ABC transporter domains.
  • 结构域The PDZ-binding motif mediates interactions with GOPC and with the SLC4A7, SLC9A3R1/EBP50 complex.
  • 翻译后修饰Phosphorylated; activates the channel. It is not clear whether PKC phosphorylation itself activates the channel or permits activation by phosphorylation at PKA sites.
    Ubiquitinated, leading to its degradation in the lysosome. Deubiquitination by USP10 in early endosomes, enhances its endocytic recycling.
  • 细胞定位Early endosome membrane.
  • Information by UniProt

Recombinant Human CFTR protein 图像

  • 12.5% SDS-PAGE showing ab114246 at approximately 36.63kDa stained with Coomassie Blue.

Recombinant Human CFTR protein (ab114246)参考文献

ab114246 has not yet been referenced specifically in any publications.

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