外分泌胰腺囊性纤维化。
The cystic fibrosis of exocrine pancreas.
机构信息
Pediatric Gastroenterology, Hadassah University Hospital, Jerusalem 91240, Israel.
出版信息
Cold Spring Harb Perspect Med. 2013 May 1;3(5):a009746. doi: 10.1101/cshperspect.a009746.
Abstract
The cystic fibrosis transmembrane conductance regulator (CFTR) protein is highly expressed in the pancreatic duct epithelia and permits anions and water to enter the ductal lumen. This results in an increased volume of alkaline fluid allowing the highly concentrated proteins secreted by the acinar cells to remain in a soluble state. This work will expound on the pathophysiology and pathology caused by the malfunctioning CFTR protein with special reference to ion transport and acid-base abnormalities both in humans and animal models. We will also discuss the relationship between cystic fibrosis (CF) and pancreatitis, and outline present and potential therapeutic approaches in CF treatment relevant to the pancreas.
摘要
囊性纤维化跨膜电导调节因子（CFTR）蛋白在胰腺导管上皮细胞中高度表达，允许阴离子和水进入导管腔。这导致碱性液体体积增加，使腺泡细胞分泌的高浓度蛋白质保持溶解状态。这项工作将详细阐述功能失调的 CFTR 蛋白引起的病理生理学和病理学，特别参考人类和动物模型中的离子转运和酸碱异常。我们还将讨论囊性纤维化（CF）和胰腺炎之间的关系，并概述与胰腺相关的 CF 治疗的现有和潜在治疗方法。
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