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. 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 .
Application6CFSMEo- human CF submucosal gland epithelial cell line was derived from a cystic fibrosis patient who was compound heterozygote for the ΔF508 and Q2X CFTR mutations.
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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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RESEARCH USE ONLY. This product is regulated in France when intended to be used for scientific purposes, including for import and export activities (Article L 1211-1 paragraph 2 of the Public Health Code). The purchaser (i.e. enduser) is required to obtain an import authorization from the France Ministry of Research referred in the Article L1245-5-1 II. of Public Health Code. By ordering this product, you are confirming that you have obtained the proper import authorization.
