製品の概要

  • 製品名Recombinant Human CFTR protein
  • タンパク質長Protein fragment

製品の詳細

  • 由来Recombinant
  • 由来Wheat germ
  • アミノ酸配列
    • アクセッション番号P13569
    • 生物種Human
    • 配列YQIIRRTLKQAFADCTVILCEHRIEAMLECQQFLVIEENKVRQYDSIQKL LNERSLFRQAISPSDRVKLFPHRNSSKCKSKPQIAALKEETEEEVQDTRL
    • 分子量37 kDa including tags
    • 領域1381 to 1480

特性

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.

  • アプリケーション

    ELISA

    SDS-PAGE

    Western blot

  • 製品の状態Liquid
  • 備考Protein concentration is above or equal to 0.05 µg/µl
  • Concentration information loading...

前処理および保存

  • 保存方法および安定性

    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

関連情報

  • 別名
    • 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
  • 機能Involved in the transport of chloride ions. May regulate bicarbonate secretion and salvage in epithelial cells by regulating the SLC4A7 transporter.
  • 組織特異性Found on the surface of the epithelial cells that line the lungs and other organs.
  • 関連疾患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.
  • 配列類似性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.
  • ドメインThe PDZ-binding motif mediates interactions with GOPC and with the SLC4A7, SLC9A3R1/EBP50 complex.
  • 翻訳後修飾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.
  • 細胞内局在Early endosome membrane.
  • Information by UniProt

Recombinant Human CFTR protein 画像

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

Recombinant Human CFTR protein (ab114246) 使用論文

ab114246 has not yet been referenced specifically in any publications.

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