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 .
CFBE41o- 6.2 WT-CFTR Human CF Bronchial Epithelial Cell line is a subclone derived from the electroporation of the parental CFBE41o- cell line with an Epstein-Barr virus (EBV)-based episomal pCEP4β vector containing the 6.2 kb full length wtCFTR cDNA and a Hygromycin B resistance gene . The 6.2kb wtCFTR cDNA contains both the 5’ and 3’ UTR sequences that are known to affect translational efficiency and mRNA stability. The parental CFBE41o- is a CF human bronchial epithelial cell line, derived from a CF patient homozygous for the ΔF508 CFTR mutation and immortalized with the origin-of-replication defective SV40 plasmid (pSVori-) .
In CFBE41o- 6.2 WT-CFTR cells, the levels of vector-driven wt-CFTR mRNA were found to be significantly higher than endogenous CFTR mRNA levels in normal 16HBE14o- bronchial epithelial cell (Cat. No. SCC150). However, cAMP-dependent Cl currents generated in CFBE41o- 6.2 WT-CFTR cells were not as high as those observed in 16HBE14o- cells, which express endogenous CFTR. Established CF bronchial epithelial cell lines that are complemented with either wild-type or ΔF508CFTR mRNA would help provide insights into the relationship between transgene-derived CFTR mRNA expression and rescue of cAMP-dependent Cl transport function.
ApplicationCFBE41o- 6.2 WT-CFTR human CF bronchial epithelial cell line is used to study the relationship between CFTR mRNA expression and Cl transport function.
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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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