Cystic fibrosis epithelial cells

D. C. Gruenert, C. B. Basbaum, and J. H. Widdicombe. Long-term culture of normal and cystic fibrosis epithelial cells grown under serum-free condition. In Vitro Cell Dev Biol 26 411-418 (1990). 

Egan ME, Glockner-Pagel J, Ambrose C, Cahill PA, Pappoe L, Balamuth N, Cho E, Canny S, Wagner CA, Geibel J, Caplan MJ. Calcium-pump inhibitors induce functional surface expression of Delta F508-CFTR protein in cystic fibrosis epithelial cells. Nat. Med. 2002 8 485-192. 

Norez C, Noel S, Wilke M, Bijvelds M, Joma H, Melin P, DeJonge H, Becq F. Rescue of functional delF508-CFTR channels in cystic fibrosis epithelial cells by the alpha-glucosidase inhibitor miglustat. FEES Lett. 2006 580 2081-2086. 

Imundo L, Barasch J, Price A, Al-Awqati Q. Cystic fibrosis epithelial cells have a receptor for pathogenic bacteria on their apical surface. Proc Natl Acad Sci USA 1995 92 3019. 

KeUey TJ, Elmer HL, Corey DA. Reduced SmadS protein expression and altered transforming growth factor-betal-mediated signaling in cystic fibrosis epithelial cells. Am J Respir Cell Mol Biol 2001 25 732. 

FIGURE 13-1. Electrolyte transport in the airway epithelial cell. Ca, calcium cAMP, cyclic-3, 5 -adenosine monophosphate Cl, chloride Na, sodium K potassium. (From Milavetz G, Smith JJ. Cystic fibrosis. In DiPiro JT, Talbert RL, Yee GC, et al, (eds.) Pharmacotherapy A Pathophysiologic Approach. 6th ed. New York McGraw-Hill 2005 592, with permission.)... 

A second approach involves direct injection/administration of the nucleic-acid-containing vector to the target cell, in situ in the body. Examples of this approach have included the direct injection of vectors into a tumour mass, as well as aerosol administration of vectors (e.g. containing the cystic fibrosis gene) to respiratory tract epithelial cells. 

The cystic fibrosis (cf) gene was first identified in 1989. It codes for CFTR, a 170 kDa protein that serves as a chloride channel in epithelial cells. Inheritance of a mutant cftr gene from both parents results in the cystic fibrosis phenotype. While various organs are affected, the most severely affected are the respiratory epithelial cells. These cells have, unsurprisingly, become the... 

Stutts MJ, Cotton CU, Yankaskas JR, Cheng E, Knowles MR, Gatzy JT, Boucher RC (1985) Chloride uptake into cultured airway epithelial cells from cystic fibrosis patients and normal individuals. Proc Natl Acad Sci USA 82(19) 6677-6681. 

Scholte BJ, Kansen M, Hoogeveen AT, Willemse R, Rhim JS, van der Kamp AW, Bijman J (1989) Immortalization of nasal polyp epithelial cells from cystic fibrosis patients. Exp Cell Res 182(2) 559—571. 

Ehrhardt C, Collnot EM, Baldes C, Becker U, Laue M, Kim KJ, Lehr CM (2006) Towards an in vitro model of cystic fibrosis small airway epithelium Characterisation of the human bronchial epithelial cell line CFBE41o-. Cell Tissue Res 323(3) 405-415. 

Zeitlin, P. L., L. Lu, J. Rhim, G. Cutting, G. Stetten, K. A. Kieffer, R. Craig, and W. B. Guggino. 1991. A cystic fibrosis bronchial epithelial cell line Immortalization by adeno-12-SV40 infection. Am J Respir Cell Mol Biol 4 313-9. 

Cozens, A. L., M. J. Yezzi, L. Chin, E. M. Simon, W. E. Finkbeiner, J. A. Wagner, and D. C. Gruenert. 1992. Characterization of immortal cystic fibrosis tracheobronchial gland epithelial cells. Proc Natl Acad Sci USA 89(11) 5171—5. 

Kunzelmann, K., D. C. Lei, K. Eng, L. C. Escobar, T. Koslowsky, and D. C. Gruenert. 1995. Epithelial cell specific properties and genetic complementation in a delta F508 cystic fibrosis nasal polyp cell line. In Vitro Cell Dev Biol Anim 31(8) 617—24. 

Kunzelmann, K., E. M. Schwiebert, P. L. Zeitlin, W. L. Kuo, B. A. Stanton, and D. C. Gruenert. 1993. An immortalized cystic fibrosis tracheal epithelial cell line homozygous for the delta F508 CFTR mutation. Am J Respir Cell Mol Biol 8(5) 522-9. 

Yankaskas, J. R., and R. C. Boucher. 1990. Transformation of airway epithelial cells with persistence of cystic fibrosis or normal ion transport phenotypes. Methods Enzymol 192 565-71. 

The gene responsible for CF codes for the cystic fibrosis transmembrane conductance regulator (CFTR), which is a chloride channel expressed on the surface of epithelial cells that line the affected organs. 

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