Baxter P
Department of Paediatrics, Children's Hospital, Sheffield.
J R Coll Physicians Lond. 1990 Apr;24(2):98-100.
Cystic fibrosis primarily affects tissues containing secretory epithelia, but not all of them become diseased. The distribution of clinical disease corresponds closely to the effect on net anion-linked water secretion, which is reduced across epithelia of endodermal and mesodermal origin but not those of ectodermal origin. Although the gene is expressed in all secretory epithelia, its effects on water secretion are bypassed in those of ectodermal origin.
囊性纤维化主要影响含有分泌上皮的组织,但并非所有这些组织都会患病。临床疾病的分布与对净阴离子相关水分泌的影响密切相关,在内胚层和中胚层来源的上皮中这种分泌减少,而外胚层来源的上皮则不然。尽管该基因在所有分泌上皮中都有表达,但其对水分泌的影响在外胚层来源的上皮中被绕过。