CFBE41o- Human CF Bronchial Epithelial Cell Line

Descrição detalhada : 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 (?F508) in the CFTR protein. This mutation accounts for ~66% of all CF alleles. CFBE41o- is a CF human bronchial epithelial cell line, derived from a CF patient homozygous for the ?F508 CFTR mutation and immortalized with the origin-of-replication defective SV40 plasmid (pSVori-) . CFBE14o- displays all ion transport properties characteristic of cystic fibrosis such as defective cAMP-dependent chloride transport and intact calcium-dependent chloride transport. Under appropriate culture conditions, CFBE41o- forms tight junctions to give a polarized epithelium . The CFBE41o- cell line has also been used to generate subclones complemented with wild-type or ?F508CFTR that possess differing levels of transgene-derived CFTR mRNA expression . These complemented CFBE41o- subclones (Cat. No. SCC158 - SCC161) suggest a direct correlation in CFTR mRNA expression levels to the number of active CFTR channels .
Sinónimos : CFBE41o, CF41o, CFBE
Armazenamento : liquid N2 (-196°C)
Embalagem : 1X1EA