Anti -Cystic Fibrosis Transmembrane Conductance Regulator (CFTR)
Pricing
For pricing information, USA customers sign in. Outside USA? Please contact your distributor for pricing. Specifications
| Clone |
Host |
Grade |
Applications |
| Polyclonal |
Rabbit |
Affinity Purified |
E B |
|
| CFTR (ABC35, ABCC7, CBAVD, CF, dj760C5.1, MRP7, TNR-CFTR) is a member of the ATPbinding cassette (ABC) transporter superfamily. Mutations in ABC genes have been linked to many diseases. CFTR is a plasma membrane cyclic AMP activated chloride channel that is expressed in the epithelial cells of the lung and several other organs (1,2). It mediates the secretion of Cland also regulates several channels including the epithelial sodium channel (ENaC), K+ channels , ATP release mechanisms, anion exchangers, sodium bicarbonate transporters and aquaporin water channels (3,4,5,6,7,8 9,10). Mutations in the CFTR gene cause cystic fibrosis, a disease that is characterized by exocrine pancreatic insufficiency, increase in sweat gland NaCl, male infertility and airway disease (1,2,11). Intracellular trafficking regulates the number of CFTR molecules at the cell surface, which in part regulates Cl- secretion. Deletion of phenylalanine 508 (deltaF508) is the most common mutation in CF patients. This mutation results in retention in the ER, where ER quality control mechanisms target the deltaF508 mutant to the proteosome for degradation (12–15). Therefore, disruption of CFTR trafficking leads to disregulation of Cl- secretion at the plasma membrane of epithelial cells. | | | Catalog # | C9040-05A | | Applications | Suitable for use in ELISA and Western Blot. Other applications not tested. | | Recommended Dilution | Western blot 1:1000 | | Optimal dilutions to be determined by the researcher. | | Storage and Stability | May be stored at 4°C for short-term only. For long-term storage, store at -20°C. Aliquots are stable for at least 12 months at -20°C. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Further dilutions can be made in assay buffer. | | CAS Number | n/a | | Clone Type | Polyclonal | | Host | Rabbit | | Source | Human | | Concentration | As reported | | Form | Supplied as a liquid in 10mM sodium HEPES, pH 7.5, 150mM sodium chloride, 0.1mg/ml BSA and 50% glycerol. | | Purity | Purified by Protein A ffinity chromatography. | | Immunogen | Synthetic peptide (KLH-coupled) corresponding to amino acids near the amino terminus of human CFTR. | | Specificity | Detects endogenous levels of total CFTR protein. Species Crossreactivity: Human. monkey, mouse and rat. | | | Important Note | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications without the expressed written authorization of United States Biological. |
External Links
|
