Overview

  • Product name
    Recombinant Human CFTR protein
  • Protein length
    Protein fragment

Description

  • Nature
    Recombinant
  • Source
    Wheat germ
  • Amino Acid Sequence
    • Accession
    • Species
      Human
    • Sequence
      YQIIRRTLKQAFADCTVILCEHRIEAMLECQQFLVIEENKVRQYDSIQKL LNERSLFRQAISPSDRVKLFPHRNSSKCKSKPQIAALKEETEEEVQDTRL
    • Molecular weight
      37 kDa including tags
    • Amino acids
      1381 to 1480

Specifications

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.

  • Applications

    ELISA

    SDS-PAGE

    Western blot

  • Form
    Liquid
  • Additional notes
    Protein concentration is above or equal to 0.05 µg/µl
  • 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
    • 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 conductance regulator ATP binding cassette sub family C member 7
    • Cystic Fibrosis Transmembrane Regulator
    • dJ760C5.1
    • MRP 7
    • MRP7
    • TNR CFTR
    see all
  • Function
    Involved in the transport of chloride ions. May regulate bicarbonate secretion and salvage in epithelial cells by regulating the SLC4A7 transporter.
  • Tissue specificity
    Found on the surface of the epithelial cells that line the lungs and other organs.
  • Involvement in disease
    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.
  • Sequence similarities
    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.
  • Domain
    The PDZ-binding motif mediates interactions with GOPC and with the SLC4A7, SLC9A3R1/EBP50 complex.
  • Post-translational
    modifications
    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.
  • Cellular localization
    Early endosome membrane.
  • Information by UniProt

Recombinant Human CFTR protein images

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

References for Recombinant Human CFTR protein (ab114246)

ab114246 has not yet been referenced specifically in any publications.

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

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