Molecular genetics of cystic fibrosis

囊性纤维化是一种威胁生命的肺部和消化系统疾病。它是高加索人群中最常见的单基因疾病，大部分病死率是由慢性肺部感染引起的，最明显的是细菌病原体铜绿假单胞菌。尽管囊性纤维化基因是在10多年前被发现的，但我们仍然没有明确的迹象表明，CF基因的缺陷如何导致细菌感染的易感性，并导致肺部炎症。我们的研究通过检测铜绿假单胞菌感染后基因表达的变化来解决这个问题，从而为我们提供了针对Cf基因介导的炎症的治疗途径。

Cystic fibrosis is a life-threatening disease of the lungs and digestive system. It is the most common single gene disorder of Caucasian populations and most of the moratility is caused by the presence of chronic lung infections, most notably with the bacterial pathogen, Pseudomonas aeruginosa. Despite the cystic fibrosis gene being discovered over 10 years ago we still have no clear indication as to how defects in the CF gene cause susceptibility to bacterial infections, and result in the inflammation of the lung. Our studies address this issue by examining thechanges of gene expression in response to infection with Pseudomonas aeruginosa and therefore provide us with routes to therapies which are targetted against CF gene mediated inflammation.