General descriptionCystic Fibrosis (CF) is a lethal autosomal recessive disease caused by mutations in the CF transmembrane conductance regulator (CFTR) gene which functions as a cAMP-activated and phosphorylated-regulated Cl channel. The predominant mutation in the CFTR gene is a trinucleotide deletion that results in loss of a phenylalanine at amino acids 508 (ΔF508) in the CFTR protein. This mutation accounts for ~66% of all CF alleles .
CFTE29o- is a human CF tracheal epithelial cell line, derived from a 21-yr old male CF patient homozygous for the ΔF508 CFTR mutation and immortalized with the origin-of-replication defective SV40 plasmid (pSVori-) . The cell line express keratin, indicating an epithelial cell origin, along with a calcium-dependent cell adhesion protein, cellCAM 120/80 , and ZO-1 indicating the ability to form tight junctions. CFTE29o- displays ion transport properties characteristic of cystic fibrosis such as defective cAMP-dependent chloride transport. Upon treatment with the calcium ionophore, ionomycin, CFTE29o- secretes chloride, albeit at reduced levels relative to normal cells. The cells express CFTR mRNA, and small amounts of CFTR protein as assessed by Western blot hybridization . Karyotype analysis indicates that 70% of the cells contain two copies of chromosome 7 .
CFTE29o- is a useful model for cystic fibrosis and for studying chloride ion transport and ion transport in human airways. The cells are useful for testing candidate chemotherapeutic drugs for their potential as treatments for ameliorating CF pathologies.
ApplicationCFTE29o- human cystic fibrosis tracheal epithelial cell line is a useful model for cystic fibrosis research and for studying chloride ion transport and ion transport in human airways.
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Biochem/physiol ActionsEpithelial Cells
Preparation NoteStore in liquid nitrogen. The cells can be cultured for at least 10 passages after initial thawing without significantly affecting the cell marker expression and functionality.
Analysis Note• Each vial contains ≥ 1X10⁶ viable cells.
• Cells are tested by PCR and are negative for HPV-16, HPV-18, Hepatitis A, C, and HIV-1 & 2 viruses as assessed by a Human Essential CLEAR panel by Charles River Animal Diagnostic Services.
• Cells are negative for mycoplasma contamination.
• Each lot of cells is genotyped by STR analysis to verify the unique identity of the cell line.
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RESEARCH USE ONLY. This product is regulated in France when intended to be used for scientific purposes, including for import and export activities (Article L 1211-1 paragraph 2 of the Public Health Code). The purchaser (i.e. enduser) is required to obtain an import authorization from the France Ministry of Research referred in the Article L1245-5-1 II. of Public Health Code. By ordering this product, you are confirming that you have obtained the proper import authorization.
