General description
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 .
Cell Line Description
Epithelial Cells
Application
6CFSMEo- 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.
This product is intended for sale and sold solely to academic institutions for internal academic research use per the terms of the “Academic Use Agreement” as detailed in the product documentation. For information regarding any other use, please contact licensing@emdmillipore.com.
Quality
• 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.
Storage and Stability
Store 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.
Disclaimer
This product contains genetically modified organisms (GMO). Within the EU GMOs are regulated by Directives 2001/18/EC and 2009/41/EC of the European Parliament and of the Council and their national implementation in the member States respectively. This legislation obliges {HCompany} to request certain information about you and the establishment where the GMOs are being handled. Click here for Enduser Declaration (EUD) Form.
Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
Shipping Information:
Hazmat Fee: $65.00 + applicable shipping charges are added after the order is placed.
More Information: https://cenmed.com/shipping-returns
- UPC:
- 51161541
- Condition:
- New
- Availability:
- 3-5 Days
- Weight:
- 1.00 Ounces
- HazmatClass:
- Yes
- MPN:
- SCC157
