Harris A
Institute of Molecular Medicine, Oxford University, John Radcliffe Hospital, UK.
Ann N Y Acad Sci. 1999 Jun 30;880:17-30. doi: 10.1111/j.1749-6632.1999.tb09506.x.
The pancreatic duct cell is central to the etiology of cystic fibrosis (CF) and is the site where pathology commences in utero. We have evaluated expression of the cystic fibrosis transmembrane conductance regulator gene (CFTR) through human development and shown it to be expressed from the early mid-trimester, with highest levels in the most distal portion of the developing duct system and in centroacinar cells. The precise cause of pancreatic destruction in CF is thought to be the obstruction of pancreatic ducts with inspissated secretions. We have shown that the MUC6 mucin is a significant component of the material that obstructs the ducts and that the MUC6 gene shows a very similar pattern of expression to that of CFTR in the developing pancreas. These observations provide a starting point for investigating how mutations in CFTR lead to obstruction of the pancreatic ducts in CF.
胰腺导管细胞在囊性纤维化（CF）的病因学中起核心作用，是子宫内病理过程开始的部位。我们评估了囊性纤维化跨膜传导调节因子基因（CFTR）在人类发育过程中的表达情况，结果显示该基因从中孕期早期开始表达，在发育中的导管系统最远端部分以及腺泡中心细胞中表达水平最高。CF中胰腺破坏的确切原因被认为是浓稠分泌物阻塞胰腺导管。我们已经表明，MUC6粘蛋白是阻塞导管物质的重要组成部分，并且MUC6基因在发育中的胰腺中的表达模式与CFTR非常相似。这些观察结果为研究CFTR突变如何导致CF患者的胰腺导管阻塞提供了一个起点。
