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Cystic 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- 4.7 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 pCEPβ vector containing the 4.7 kb wild-type CFTR open reading frame (ORF) cDNA and a Hygromycin B resistance gene . 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.,官网链接:https://www.sigmaaldrich.cn/product/mm/scc158
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