Anti-CFTR antibody - Carboxyterminal end (ab56058)
Key features and details
- Rabbit polyclonal to CFTR - Carboxyterminal end
- Reacts with: Recombinant fragment
- Isotype: IgG
Overview
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Product name
Anti-CFTR antibody - Carboxyterminal end
See all CFTR primary antibodies -
Description
Rabbit polyclonal to CFTR - Carboxyterminal end -
Host species
Rabbit -
Species reactivity
Reacts with: Recombinant fragment
Predicted to work with: Sheep, Human -
Immunogen
A synthetic peptide corresponding to C-terminal residues of human CFTR (Cystic fibrosis transmembrane conductance regulator)
Properties
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Form
Liquid -
Storage instructions
Shipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid freeze / thaw cycles. -
Storage buffer
pH: 7.2
Preservative: 0.01% Sodium azide
Constituents: 50% Glycerol (glycerin, glycerine), PBS -
Concentration information loading...
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Purity
Immunogen affinity purified -
Clonality
Polyclonal -
Isotype
IgG -
Research areas
Associated products
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Compatible Secondaries
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Isotype control
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Recombinant Protein
Target
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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
modificationsPhosphorylated; 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
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Database links
- Entrez Gene: 1080 Human
- Entrez Gene: 443347 Sheep
- Omim: 602421 Human
- SwissProt: P13569 Human
- SwissProt: Q00555 Sheep
- Unigene: 489786 Human
- Unigene: 621460 Human
- Unigene: 661104 Human
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Alternative names
- ABC 35 antibody
- ABC35 antibody
- ABCC 7 antibody
see all
Datasheets and documents
References (1)
ab56058 has been referenced in 1 publication.
- Riquelme SA et al. Cystic Fibrosis Transmembrane Conductance Regulator Attaches Tumor Suppressor PTEN to the Membrane and Promotes Anti Pseudomonas aeruginosa Immunity. Immunity 47:1169-1181.e7 (2017). PubMed: 29246444