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. In CF, altered Cl- transport and secretion results in the production of thick and viscous mucus that can damage many of the body’s organs. Tracheobronchial submucosal glands secrete mucins and antimicrobial substances that keep the airways sterile along with fluids that help hydrate airway surfaces. The relationship between CF and mucus secretion is unclear and require further investigations .6CFSMEo- is a human tracheobronchial submucosal gland epithelial cell line isolated from an individual with CF, who was compound heterozygote for the ΔF508 and Q2X CFTR mutations . ΔF508 mutation 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 . Q2X mutation is a rare CF mutation in exon 1 of the CFTR gene in which the second codon (CAG) is mutated into the stop codon UAG. The 6CFSMEo- cell line was immortalized with the origin-of-replication defective SV40 plasmid (pSVori-) . 6CFSMEo- retains the characteristic cobblestone morphology of epithelial cells along with cytokeratin expression and the ability to form tight junctions. The cell line expresses vestigial amounts of CFTR mRNA transcripts but does not express detectable levels of CFTR protein . 6CFSMEo- lacks cAMP-induced Cl- currents .，官网链接：https://www.sigmaaldrich.cn/product/mm/scc157 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。

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