Clinical cystic fibrosis

December 2003
Thorax;Dec2003 Supplement 3, Vol. 58, piii63
Academic Journal
Poor genotype-phenotype correlation in cystic fibrosis (CF) leads to the hypothesis that secondary genetic factors exist which impact on lung disease. TLR4 is a pattern recognition receptor which binds lipopolysaccharide, a constitutive element of Pseudomonos cell wall, and activates the innate immune system promoting proinflammatory cytokine release-a putative mechanism for parenchymcil lung damage in CF. Patients with CF become chronically infected with Pseudomonas aeruginoso with many phenotypic variants on culture.


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