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 . 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.，官网链接：https://www.sigmaaldrich.cn/product/mm/scc162 默克 科研、开发、生产。 作为生命科学行业的全球领先供应商，我们致力于为科研、生物技术开发和生产，以及制药药物疗法开发和生产提供各类解决方案和服务。

非质量问题不退不换