This work is concerned with Cystic Fibrosis (CF), the most common fatal genetic disease in the Caucasian population. The decade of the 1980s was one of spectacular progress in understanding the genetic and molecular basis of CF. The research breakthroughs of the decade began with the first fundamental insights, published in 1981–1983, into the basic cellular pathophysiology of CF with demonstrations of alteredion transport in specialized exocrine epithelial tissues (1–3). Research progress shifted into a triumph of “reverse genetics,” using restriction-fragment-length polymorphism DNA technology (4), with the localization of the CF gene to a region of chromosome 7 (5–7). Understanding, accelerated by an explosion of in vitro methodologies for epithelial cell culture and transformation, allowed controlled biochemical and physiological studies (8–11); these focused, with increasing precision, on the molecular pathology of distal steps in the regulatory pathways for epithelial ion transport (12–19). Finally, the “end of the beginning” occurred in late 1989with one of the great achievements of molecular genetics, the isolation and cloning of the CF gene (20). As a result, we now have a CF gene product, the cystic fibrosis transmembrane regulator (CFTR), possessing predicted amino acid sequence, suggested tertiary structure, and possible transmembrane transport function (21).
KeywordsCatheter Mold Pseudomonas Aspergillus Sinusitis