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2006, Cilt 20, Sayı 1, Sayfa(lar) 073-078
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THE MOLECULAR BIOLOGY AND PATHOGENESIS OF CYSTIC FIBROSIS
Çağla BOZKURT-GÜZEL, A. Alev GERÇEKER
İstanbul Üniversitesi Eczacılık Fakültesi, Farmasötik Mikrobiyoloji Anabilim Dalı, İstanbul
Keywords: Cystic fibrosis, molecular biology, pathogenesis, cystic fibrosis transmembrane conductance regulator (CFTR), Pseudomonas aeruginosa

Cystic fibrosis which is seen in every 1 in 2500 person especially in Caucasian origin is inherited as an autosomal recessive character. This disease is caused by the mutations in the gene coding cystic fibrosis transmembrane conductance regulator (CFTR). Physiopathologic changes that are seen in the lungs of cystic fibrosis patients cause infections triggered by various microorganisms. Pseudomonas aeruginosa triggered infections cause serious loss in pulmonary function in more than 80 % of the cystic fibrosis patients and end with early deaths. On the other hand, P. aeruginosa uses CFTR protein as a binding site to enter epithelial cells, and the absence of functional CFTR protein in cystic fibrosis patients causes a failure in innate immunity leading to initiation of a bacterial infection in the airway. In this paper, the molecular biology and the function of cystic fibrosis gene and its product CFTR protein, mutations in the gene that cause cystic fibrosis, the pathogenesis of the disease and the contribution of P. aeruginosa to the pathogenesis of cystic fibrosis are reviewed.

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