Stern M, Geddes D
Royal Brompton Hospital, London.
Br J Hosp Med. 1996;55(5):237-40.
Cystic fibrosis (CF) primarily affects epithelial-lined organs, the most important being the lungs. Much of the known pathogenesis of the disease has been elucidated by the identification of the gene mutated in CF which encodes the cystic fibrosis transmembrane conductance regulator, a chloride channel in the apical membrane of epithelial cells of the respiratory and intestinal tracts.
囊性纤维化（CF）主要影响内衬上皮的器官，其中最重要的是肺部。通过鉴定CF中发生突变的基因，已经阐明了该疾病许多已知的发病机制，该基因编码囊性纤维化跨膜传导调节因子，它是呼吸道和肠道上皮细胞顶端膜中的一种氯离子通道。
