The Cystic Fibrosis Intestine
The Cystic Fibrosis Intestine
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DOI：
10.1101/cshperspect.a009753
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发表时间：
2013-09-01
影响因子：
5.4
通讯作者：
Borowitz, Drucy
中科院分区：
文献类型：
--
作者：
De Lisle, Robert C.;Borowitz, Drucy
囊性纤维化（CF）的临床表现是由囊性纤维化跨膜调节蛋白（CFTR）功能障碍引起的。由于呼吸道CFTR功能障碍，大多数CF患者的寿命有限。然而，胃肠道CFTR功能障碍发生在个体发育早期，并且存在于所有患者中，无论基因型如何。引起气道疾病的同样的三位一体的病理生理阻塞、感染和炎症也会引起肠道疾病。本文介绍了CFTR功能障碍对肠道组织和腔内环境的影响。CF的小鼠模型极大地促进了我们对CF的GI表现的认识，可以直接应用于对人类CF疾病的认识。
The clinical manifestations of cystic fibrosis (CF) result from dysfunction of the cystic fibrosis transmembrane regulator protein (CFTR). The majority of people with CF have a limited life span as a consequence of CFTR dysfunction in the respiratory tract. However, CFTR dysfunction in the gastrointestinal (GI) tract occurs earlier in ontogeny and is present in all patients, regardless of genotype. The same pathophysiologic triad of obstruction, infection, and inflammation that causes disease in the airways also causes disease in the intestines. This article describes the effects of CFTR dysfunction on the intestinal tissues and the intraluminal environment. Mouse models of CF have greatly advanced our understanding of the GI manifestations of CF, which can be directly applied to understanding CF disease in humans.