By Richard B. Moss (auth.), Richard B. Moss (eds.)
This paintings is anxious with Cystic Fibrosis (CF), the most typical deadly genetic ailment within the Caucasian inhabitants. the last decade of the Eighties used to be one in every of magnificent development in realizing the genetic and molecu lar foundation of CF. The study breakthroughs of the last decade begun with the 1st primary insights, released in 1981-1983, into the fundamental mobile pathophysiology of CF with demonstrations of altered ion delivery in spe cialized exocrine epithelial tissues (1-3). examine growth shifted right into a triumph of "reverse genetics," utilizing restriction-fragment-Iength polymor phism DNA expertise (4), with the localization of the CF gene to a zone of chromosome 7 (5-7). realizing, speeded up by means of an explOSion of in vitro methodologies for epithelial cellphone tradition and transformation, allowed and physiological reviews (8-11); those centred, managed biochemical with expanding precision, at the molecular pathology of distal steps within the regulatory pathways for epithelial ion shipping (12-19). ultimately, the "end of the start" happened in overdue 1989 with one of many nice achievements of molecular genetics, the isolation and cloning of the CF gene (20). consequently, we have a CF gene product, the cystic fibrosis transmembrane regulator (CFfR), owning anticipated amino acid series, prompt tertiary constitution, and attainable transmembrane shipping functionality (21). those remarkable advancements have set the degree for the following around of advances, which without doubt will comprise: 1.
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Extra resources for Cystic Fibrosis: Infection, Immunopathology, and Host Response
W. (1975), J. Med. Microbiol. 8,513-522. 77. Joris, L. and Quinton, P. (1987), Med. Sci. Res. 15,855-856. 78. Wood, R. E. (1984), Cystic Fibrosis (Taussig, L. , New York, pp. 434-460. 79. , Scambler, P. , Bell, G. , Hawley, K M. , Lench, N. , Bates, G. , Kruyer, H. , Frederick, P. , Watson, E. , and Wainwright, B. J. (1987), Nature 326, 840- 845. 80. , Webb, L. , and Hobgood, C. D. (1985), Science 228, 990-993. The Relationship Between Atopy and Cystic Fibrosis Robert W. Wilmott Children's Hospital Medical Center, Eiland and Bethesda Avenues, Cincinnati, OH 45229 INTRODUCTION Cystic fibrosis (CF) is a multisystem disease affecting exocrine gland function.
60. 61. 62. 27 Gumbiner, B. (1987), Am. J. Physiol. 253, C749-C758. Almers, W. and Sterling, C. (1984), J. Membr. Bioi. 77,169-186. Frizzell, R. , and Schultz, S. G. (1979), Am. J. Physiol. 236,1-8. Welsh, M. J. (1987), Physiol Rev. 67,1143-1184. Powell, D. W. (1986), Physiology of Membrane Disorders, 2nd Ed. (Andreoli, T. , Hoffman, J. , Fannestil, D. , and Schultz, S. ), Plenum Press, New York, pp. 559-583. Reddy, M. M. and Quinton, P. M. (1989), Am. J. Physiol. C727-C735. Willumsen,N. J. and Boucher, R.
Large deletions might be expected in a gene of this size; their absence in the CF population could mean that they are highly lethal, suggesting that the CFTR protein retains a crucial function in CF individuals. (Depending on how one assigns functions, this possibility is not inconsistent with the idea that more than one function is lost in most types ofCF. ) It is risky, but probably obligatory, to conclude a review of this kind by speculating on the possibilities for ameliorating or curing CF.