急性威尔逊病中的肝衰竭和肝细胞损伤涉及CD95（APO-1/Fas）介导的细胞凋亡。

Hepatic failure and liver cell damage in acute Wilson's disease involve CD95 (APO-1/Fas) mediated apoptosis.

作者信息

Strand S, Hofmann W J, Grambihler A, Hug H, Volkmann M, Otto G, Wesch H, Mariani S M, Hack V, Stremmel W, Krammer P H, Galle P R

机构信息

University Hospital, Department of Gastroenterology, Heidelberg, FRG.

出版信息

Nat Med. 1998 May;4(5):588-93. doi: 10.1038/nm0598-588.

DOI:10.1038/nm0598-588

PMID:9585233

Abstract

Wilson's disease can result in fulminant liver failure due to hepatic copper overload. The CD95 system mediates apoptosis and has been demonstrated to be involved in liver disease. In this study CD95 mediated apoptosis was investigated in patients with fulminant hepatic failure in the course of Wilson's disease and in an in vitro model of copper treated human hepatoma cells. In patients, hepatic expression of CD95 and CD95L mRNA and apoptosis were detected. Copper overload in vitro resulted in hepatocytic apoptosis which could be reduced with a neutralizing anti-CD95L antibody. Copper treatment of hepatocytes results in activation of the CD95 system and induction of apoptosis which is operative during the course of hepatic failure in acute Wilson's disease.

摘要

威尔逊氏病可因肝脏铜过载导致暴发性肝衰竭。CD95系统介导细胞凋亡，且已被证明与肝脏疾病有关。在本研究中，对威尔逊氏病病程中暴发性肝衰竭患者以及铜处理的人肝癌细胞体外模型中的CD95介导的细胞凋亡进行了研究。在患者中，检测了CD95和CD95L mRNA的肝脏表达及细胞凋亡情况。体外铜过载导致肝细胞凋亡，而使用中和性抗CD95L抗体可使其减少。肝细胞的铜处理导致CD95系统激活并诱导细胞凋亡，这在急性威尔逊氏病肝衰竭过程中起作用。