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堵塞引起的。我们对蛋白塞进行了蛋白质组学分析以阐明其形成机制。蛋白塞取自3名儿科患者。胆总管囊肿。采用十二烷基硫酸钠-聚丙烯酰胺凝胶电泳法分离蛋白质。样品共有的凝胶带被切除用于质谱分析。质谱与NCBI数据库进行比较，进行蛋白鉴定。蛋白塞样品的凝胶带以14千道尔顿居多，其次为29千道尔顿。其他四条细带与插头样本相同。鉴定出4个波段（包括14和29千道尔顿）为石石酸盐，1个波段为血清白蛋白。栓子主要由胰腺泡细胞分泌到胰液中的一种蛋白质石石酸盐组成。其机制涉及胰蛋白酶原和静石素通过胰胆管的异常结合反流进入囊肿。被激活的胰蛋白酶将可溶性石石酸分解成不溶的形式，聚集形成栓。

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.