The mechanism of liver cancer onset in Wilson disease and its prevention

肝豆状核变性（WD）是由铜在肝脏内蓄积引起的一种疾病。本研究旨在阐明WD肝损害包括癌变的发生机制，并对其预防有一定的认识。使用该模型大鼠，它揭示了铜积累和线粒体障碍诱导的肝脏从出生后的早期阶段，此外，氧化应激不能正常控制。此外，WD患者体内的氧化应激谱（OSP）分析结果表明，血清中某些脂溶性抗氧化剂水平下降，约半数患者处于低活性区，即抗氧化能力低下。这些结果表明，这些抗氧化剂的治疗可能会阻止肝脏疾病的进展，包括在WD致癌。

Wilson disease (WD) causes various disorders by copper accumulationin the liver. The purpose of this study is to elucidate the onset mechanism of the liver damage including carcinogenesis in WD, and get the knowledge about its prevention. Using the model rat, it was revealed that copper accumulated and a mitochondrial disorder was induced in the liver from an early stage after birth, in addition, oxidative stress could not been controlled normally. Furthermore, the results of oxidative stress profile (OSP) analysis of the WD patient bodies suggested that a serum level of the certain fat-solubleantioxidants were decreasing, and about half of the patients are in the low active zone which means to have low antioxidant ability. These results suggested that the treatment with these antioxidants might prevent the progress of hepatic disorders including carcinogenesis in WD.