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You are here:Home » Antibodies » Abs to Disease Markers » Anti -Cystic Fibrosis Transmembrane Conductance Regulator (CFTR)

Anti -Cystic Fibrosis Transmembrane Conductance Regulator (CFTR)

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Specifications

Clone Host Grade Applications
Polyclonal Rabbit Affinity Purified 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 (1215). Therefore, disruption of CFTR trafficking leads to disregulation of Cl- secretion at the plasma membrane of epithelial cells.
Catalog #C9040-05A
ApplicationsSuitable for use in Western Blot. Other applications not tested.
Recommended DilutionWestern Blot: 1:1000
Optimal dilutions to be determined by the researcher.
Storage and StabilityMay be stored at 4°C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20°C. Aliquots are stable for 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
Clone TypePolyclonal
HostRabbit
SourceHuman
ConcentrationNot determined
FormSupplied as a liquid in 10mM sodium HEPES, pH 7.5, 150mM sodium chloride, 0.1mg/ml BSA and 50% glycerol.
PurityPurified by Protein A ffinity chromatography.
ImmunogenSynthetic peptide corresponding to amino acids near the amino terminus of human CFTR, conjugated to KLH.
SpecificityRecognizes endogenous levels of total human CFTR protein. Species Crossreactivity: monkey, mouse and rat.
Important NoteThis 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.


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