Sakaguchi Laboratory, Department of Systems Medicine, Keio University School of Medicine Tokyo, Japan.
Front Physiol. 2012 Nov 5;3:415. doi: 10.3389/fphys.2012.00415. eCollection 2012.
Chronic pancreatitis (CP) is a progressive inflammatory disease in which the pancreatic secretory parenchyma is destroyed and replaced by fibrosis. The presence of intraductal pancreatic stone(s) is important for the diagnosis of CP; however, the precise molecular mechanisms of pancreatic stone formation in CP were left largely unknown. Cystic fibrosis transmembrane conductance regulator (CFTR) is a chloride channel expressed in the apical plasma membrane of pancreatic duct cells and plays a central role in [Formula: see text] secretion. In previous studies, we have found that CFTR is largely mislocalized to the cytoplasm of pancreatic duct cells in all forms of CP and corticosteroids normalizes the localization of CFTR to the proper apical membrane at least in autoimmune pancreatitis. From these observations, we could conclude that the mislocalization of CFTR is a cause of protein plug formation in CP, subsequently resulting in pancreatic stone formation. Considering our observation that the mislocalization of CFTR also occurs in alcoholic or idiopathic CP, it is very likely that these pathological conditions can also be treated by corticosteroids, thereby preventing pancreatic stone formation in these patients. Further studies are definitely required to clarify these fundamental issues.
慢性胰腺炎(CP)是一种进行性炎症性疾病,其中胰腺分泌实质被破坏并被纤维化所取代。胰管内存在胰腺结石对于 CP 的诊断很重要;然而,CP 中胰腺结石形成的确切分子机制仍知之甚少。囊性纤维化跨膜电导调节因子(CFTR)是一种在胰腺导管细胞顶膜表达的氯离子通道,在[Formula: see text]分泌中起核心作用。在之前的研究中,我们发现 CFTR 在所有形式的 CP 中都主要定位于胰腺导管细胞的细胞质中,而皮质类固醇至少在自身免疫性胰腺炎中可将 CFTR 的定位恢复到适当的顶膜。从这些观察结果,我们可以得出结论,CFTR 的定位错误是 CP 中蛋白质栓形成的原因,随后导致胰腺结石形成。考虑到我们观察到 CFTR 的定位错误也发生在酒精性或特发性 CP 中,很可能这些病理状况也可以用皮质类固醇治疗,从而防止这些患者发生胰腺结石形成。进一步的研究肯定是需要的,以澄清这些基本问题。