突变型囊性纤维化跨膜传导调节因子在囊性纤维化患者对肺部感染高度易感性中的作用。
Role of mutant CFTR in hypersusceptibility of cystic fibrosis patients to lung infections.
作者信息
Pier G B, Grout M, Zaidi T S, Olsen J C, Johnson L G, Yankaskas J R, Goldberg J B
机构信息
Channing Laboratory, Brigham and Women's Hospital, Harvard Medical School, Boston, MA 02115-5899, USA.
出版信息
Science. 1996 Jan 5;271(5245):64-7. doi: 10.1126/science.271.5245.64.
Abstract
Cystic fibrosis (CF) patients are hypersusceptible to chronic Pseudomonas aeruginosa lung infections. Cultured human airway epithelial cells expressing the delta F508 allele of the cystic fibrosis transmembrane conductance regulator (CFTR) were defective in uptake of P. aeruginosa compared with cells expressing the wild-type allele. Pseudomonas aeruginosa lipopolysaccharide (LPS)-core oligosaccharide was identified as the bacterial ligand for epithelial cell ingestion; exogenous oligosaccharide inhibited bacterial ingestion in a neonatal mouse model, resulting in increased amounts of bacteria in the lungs. CFTR may contribute to a host-defense mechanism that is important for clearance of P. aeruginosa from the respiratory tract.
摘要
囊性纤维化（CF）患者极易受到铜绿假单胞菌慢性肺部感染的影响。与表达野生型等位基因的细胞相比，表达囊性纤维化跨膜传导调节因子（CFTR）的ΔF508等位基因的培养人气道上皮细胞在摄取铜绿假单胞菌方面存在缺陷。铜绿假单胞菌脂多糖（LPS）核心寡糖被确定为上皮细胞摄取的细菌配体；外源性寡糖在新生小鼠模型中抑制细菌摄取，导致肺部细菌数量增加。CFTR可能有助于一种对从呼吸道清除铜绿假单胞菌很重要的宿主防御机制。
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