Key features and details
- Rabbit polyclonal to CFTR
- Suitable for: ICC/IF, IP, WB
- Reacts with: Human
- Isotype: IgG
参阅全部 CFTR 一抗
特异性Detects cystic fibrosis transmembrane conductance factor (CFTR) from cells overexpressing the human protein.
经测试应用适用于: ICC/IF, IP, WBmore details
预测可用于: Rat, Sheep, Rabbit, Guinea pig, Cow, Dog, Pig, Chimpanzee, Monkey, Non human primates, Cynomolgus monkey, Rhesus monkey, Gorilla, Elephant
存放说明Shipped 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.
存储溶液Preservative: 0.05% Sodium azide
Constituents: 99% PBS, 0.1% BSA
Concentration information loading...
纯度Immunogen affinity purified
Primary antibody说明Cystic 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.
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.
|ICC/IF||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.|
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
- ABC 35 antibody
- ABC35 antibody
- ABCC 7 antibody
ab2916 被引用在 3 文献中.
- Li W et al. CFTR inhibits the invasion and growth of esophageal cancer cells by inhibiting the expression of NF-?B. Cell Biol Int 42:1680-1687 (2018). PubMed: 30358020
- 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 . 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). PubMed: 18665014