- Datasheet
- Specific References (2)
- Protocols
Overview
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Product nameAnti-CFTR antibody
See all CFTR primary antibodies -
DescriptionRabbit polyclonal to CFTR
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Host speciesRabbit
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SpecificityDetects cystic fibrosis transmembrane conductance factor (CFTR) from cells overexpressing the human protein.
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Tested applicationsSuitable for: ICC, IP, WBmore details
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Species reactivityReacts with: Human
Predicted to work with: Rat, Sheep, Rabbit, Guinea pig, Cow, Dog, Pig, Chimpanzee, Monkey, Non human primates, Cynomolgus monkey, Rhesus monkey, Gorilla, Elephant
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Immunogen
Synthetic peptide corresponding to Human CFTR aa 103-117.
Sequence:GRIIASYDPDNKEER
Properties
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FormLiquid
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Storage instructionsShipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C or -80°C. Avoid freeze / thaw cycle.
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Storage bufferPreservative: 0.05% Sodium azide
Constituents: 99% PBS, 0.1% BSA -
Concentration information loading... -
PurityImmunogen affinity purified
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Primary antibody notesCystic Fibrosis (CF) is a common lethal genetic disease caused by mutations of the gene coding for the cystic fibrosis transmembrane conductance factor, a cAMP regulated chloride channel. Approximately 70% of all CF cases share the deletion of a phenylalanine at position 508 (delta F508) which results in abnormal chloride transport. Since the CF mutation is lethal, most often by lung and liver disease, it raises the question of why this genetic disease remains as common as it is. One possible explanation is that Salmonella typhi has been shown to use CFTR to enter intestinal epithelial cells and that delta F508 heterozygote and homozygote mice showed 86% and 100% reductions in S. typhi intestinal submucosal uptake.
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ClonalityPolyclonal
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IsotypeIgG
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Research areas
Associated products
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Compatible Secondaries
Applications
Our Abpromise guarantee covers the use of ab2916 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
| Application | Abreviews | Notes |
|---|---|---|
| ICC | Use a concentration of 1 µg/ml. | |
| IP | Use at an assay dependent concentration. | |
| WB | Use at an assay dependent concentration. Predicted molecular weight: 168 kDa. |
Target
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FunctionInvolved in the transport of chloride ions. May regulate bicarbonate secretion and salvage in epithelial cells by regulating the SLC4A7 transporter.
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Tissue specificityFound on the surface of the epithelial cells that line the lungs and other organs.
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Involvement in diseaseDefects 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 similaritiesBelongs 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. -
DomainThe PDZ-binding motif mediates interactions with GOPC and with the SLC4A7, SLC9A3R1/EBP50 complex.
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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 localizationEarly endosome membrane.
- Information by UniProt
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Database links
- Entrez Gene: 463674 Chimpanzee
- Entrez Gene: 281067 Cow
- Entrez Gene: 102130331 Cynomolgus monkey
- Entrez Gene: 492302 Dog
- Entrez Gene: 101132066 Gorilla
- Entrez Gene: 1080 Human
- Entrez Gene: 403154 Pig
- Entrez Gene: 100009471 Rabbit
see all -
Alternative names
- ABC 35 antibody
- ABC35 antibody
- ABCC 7 antibody
see all
Images
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Immunocytochemistry/ Immunofluorescence - Anti-CFTR antibody (ab2916)Immunolocalization of CFTR in HEK293 cells stably transfected with the CFTR gene using ab2916.
Protocols
References
This product has been referenced in:
- Yang X et al. High CFTR expression in Philadelphia chromosome-positive acute leukemia protects and maintains continuous activation of BCR-ABL and related signaling pathways in combination with PP2A. Oncotarget 8:24437-24448 (2017). WB . Read more (PubMed: 28445932) »
- Klein T et al. Investigation and characterization of the duct cell-enriching process during serum-free suspension and monolayer culture using the human exocrine pancreas fraction. Pancreas 38:36-48 (2009). Read more (PubMed: 18665014) »
Customer reviews and Q&As
Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"