CFBE41o- Human CF Bronchial Epithelial Cell Line人囊性纤维化支气管上皮细胞株 BioVector NTCC质粒载体菌种细胞基因保藏中心
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- 货 号：CFBE41o- Human CF Bronchial Epithelial Cell Line人囊性纤维化支气管上皮细胞株
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CFBE41o- Human CF Bronchial Epithelial Cell Line人囊性纤维化支气管上皮细胞株 BioVector NTCC质粒载体菌种细胞基因保藏中心
CFBE41o- Human CF Bronchial Epithelial Cell Line
人囊性纤维化支气管上皮细胞株
Cat No.: NTCC691982
Alternate Names • CFBE41o
• CF41o
• CFBE
Background Information 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.
CFBE41o- is a CF human bronchial epithelial cell line, derived from a CF patient homozygous for the ΔF508 CFTR mutation and immortalized with the origin-of-replication defective SV40 plasmid (pSVori-) (1,2). 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 (1).
The CFBE41o- cell line has also been used to generate subclones complemented with wild-type or ΔF508CFTR that possess differing levels of transgene-derived CFTR mRNA expression (2). These complemented CFBE41o- subclones suggest a direct correlation in CFTR mRNA expression levels to the number of active CFTR channels (1).
References:
1. Illek B, Maurisse R, Wahler L, Kunzelmann K, Fischer H, Gruenert DC. (2008) Cl transport in complemented CF bronchial epithelial cells correlates with CFTR mRNA expression levels. Cell Physiol Biochem 22(1-4): 57-68.
2. Bruscia E, Sangiuolo F, Sinibaldi P, Goncz KK, Novelli G, Gruenert DC. (2002) Isolation of CF cell lines corrected at DeltaF508-CFTR locus by SFHR-mediated targeting. Gene Ther 9(11): 683-685.
3. Goncz KK, Feeney L, Gruenert DC. (1999) Differential sensitivity of normal and cystic fibrosis airway epithelial cells to epinephrine. Br J Pharmacol 128(1): 227-233.
4. Illek B, Lei D, Fischer H, Gruenert DC. (2010) Sensitivity of chloride efflux vs. transepithelial measurements in mixed CF and normal airway epithelial cell populations. Cell Physiol Biochem 26(6): 983-990.
Applications
Application CFBE41o- human CF bronchial epithelial cell line was derived from a cystic fibrosis patient homozygous for the ΔF508 CFTR mutation.
Key Applications • Cell Culture
• Cell Based Assays
Biological Information
Cell Line Type • Epithelial Cells
【Supplier来源】BioVector NTCC Inc.
TEL：+86-010-53513060
【Website网址】 http://www.biovector.net
CFBE41o- Human CF Bronchial Epithelial Cell Line
人囊性纤维化支气管上皮细胞株
Cat No.: NTCC691982
Alternate Names • CFBE41o
• CF41o
• CFBE
Background Information 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.
CFBE41o- is a CF human bronchial epithelial cell line, derived from a CF patient homozygous for the ΔF508 CFTR mutation and immortalized with the origin-of-replication defective SV40 plasmid (pSVori-) (1,2). 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 (1).
The CFBE41o- cell line has also been used to generate subclones complemented with wild-type or ΔF508CFTR that possess differing levels of transgene-derived CFTR mRNA expression (2). These complemented CFBE41o- subclones suggest a direct correlation in CFTR mRNA expression levels to the number of active CFTR channels (1).
References:
1. Illek B, Maurisse R, Wahler L, Kunzelmann K, Fischer H, Gruenert DC. (2008) Cl transport in complemented CF bronchial epithelial cells correlates with CFTR mRNA expression levels. Cell Physiol Biochem 22(1-4): 57-68.
2. Bruscia E, Sangiuolo F, Sinibaldi P, Goncz KK, Novelli G, Gruenert DC. (2002) Isolation of CF cell lines corrected at DeltaF508-CFTR locus by SFHR-mediated targeting. Gene Ther 9(11): 683-685.
3. Goncz KK, Feeney L, Gruenert DC. (1999) Differential sensitivity of normal and cystic fibrosis airway epithelial cells to epinephrine. Br J Pharmacol 128(1): 227-233.
4. Illek B, Lei D, Fischer H, Gruenert DC. (2010) Sensitivity of chloride efflux vs. transepithelial measurements in mixed CF and normal airway epithelial cell populations. Cell Physiol Biochem 26(6): 983-990.
Applications
Application CFBE41o- human CF bronchial epithelial cell line was derived from a cystic fibrosis patient homozygous for the ΔF508 CFTR mutation.
Key Applications • Cell Culture
• Cell Based Assays
Biological Information
Cell Line Type • Epithelial Cells
【Supplier来源】BioVector NTCC Inc.
TEL：+86-010-53513060
【Website网址】 http://www.biovector.net
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