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 ΔF508-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 ΔF508 CFTR cDNA and a Hygromycin B resistance gene . The 6.2kb ΔF508CFTR cDNA contains the trinucleotide TTT at the ΔF508 locus rather than the naturally occurring CTT and thus makes it possible to differentiate between endogenous ΔF508 CFTR and plasmid derived ΔF508CFTR expression. The 6.2kb ΔF508CFTR 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-) .
Established CF bronchial epithelial cell lines that are complemented with either wild-type or ΔF508CFTR mRNA will help provide insights into the relationship between transgene-derived CFTR mRNA expression and rescue of cAMP-dependent Cl transport function.
ApplicationCFBE41o- 6.2 ΔF508-CFTR human CF bronchial epithelial cell line may be 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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