Harris A, Chalkley G, Goodman S, Coleman L
Paediatric Research Unit, United Medical School, Guy's Hospital, London, UK.
Development. 1991 Sep;113(1):305-10. doi: 10.1242/dev.113.1.305.
The specialised epithelia lining the respiratory tract, pancreatic ducts, male genital ducts and sweat gland ducts are defective in the severe inherited disease, cystic fibrosis (CF). We have looked at the expression of the CF gene in human fetal tissues to throw light on the development of function in specialised ductal epithelia and to determine the age of onset of the CF disease process. The CF gene is already seen to be transcribed in mid-trimester fetal lung, pancreas and male genital ducts. Hence, by this developmental stage, and before they are fully differentiated, these epithelia have the capability to perform important transport functions. Epithelial cell cultures derived from fetal pancreas and male genital ducts maintain expression of the CF gene in vitro and so form good models for analysing CF gene function and differentiation of these specialised epithelia.
在严重的遗传性疾病囊性纤维化（CF）中，呼吸道、胰腺导管、男性生殖管道和汗腺导管内衬的特殊上皮存在缺陷。我们研究了CF基因在人类胎儿组织中的表达情况，以了解特殊导管上皮功能的发育过程，并确定CF疾病进程的起始年龄。在孕中期胎儿的肺、胰腺和男性生殖管道中已可观察到CF基因的转录。因此，在这个发育阶段，这些上皮在完全分化之前就具备执行重要转运功能的能力。源自胎儿胰腺和男性生殖管道的上皮细胞培养物在体外维持CF基因的表达，因此成为分析CF基因功能以及这些特殊上皮细胞分化的良好模型。
