Clarification of the mechanism to cause symptoms in patients with pancreaticobiliary maljunction

胆总管囊肿的症状是由蛋白质栓引起的。我们进行蛋白质组学分析的蛋白质栓，以阐明形成机制。从三名儿科患者获得蛋白质塞。胆总管囊肿使用十二烷基硫酸钠-聚丙烯酰胺凝胶电泳分离蛋白质。切下样品共有的凝胶带用于质谱分析。将质谱与NCBI数据库进行比较以用于蛋白质鉴定。蛋白质塞样品的凝胶带主要在14千道尔顿，其次是29千道尔顿。另外四条薄带是塞子样品所共有的。其中4条（14和29千道尔顿）为石抑素，1条为血清白蛋白。胰栓主要由胰石抑素（lithostathine）组成，这是一种由胰腺腺泡细胞分泌到胰液中的蛋白质。其机制涉及胰蛋白酶原和结石抑制素通过胰胆管的异常连接进入囊肿。活化的胰蛋白酶将可溶性石抑素切割成不溶性形式，其聚集形成栓塞。

Symptoms of choledochal cysts are caused by protein plugs. We performed proteomic analysis of protein plugs to elucidate formation mechanism. Protein plugs were obtained from three pediatric patients. with choledochal cyst. Proteins were separated using sodium dodecyl sulfate-polyacrylamide gel electrophoresis. Gel bands common to the samples were excised for mass spectrometry. Mass spectra were compared with the NCBI database for protein identification. Gel bands of protein plug samples were predominant at 14 kilodaltons, followed by 29 kilodaltons. Four other thin bands were common to the plug samples. Four bands (including 14 and 29 kilodaltons) were identified as lithostathine, and one band as serum albumin. Plugs consisted mostly of lithostathine, a protein secreted by pancreatic acinar cells into pancreatic juice. The mechanism involves trypsinogen and lithostathine regurgitating into the cyst through an aberrant union of pancreaticobiliary ducts. Activated trypsin cleaves soluble lithostathine into insoluble forms that aggregate to form plugs.