SPONTANEOUS HEPATIC COPPER ACCUMULATION IN LONG-EVANS CINNAMON RATS WITH HEREDITARY HEPATITIS - A MODEL OF WILSONS-DISEASE

被引：311
|
作者：
LI, Y
TOGASHI, Y
SATO, S
EMOTO, T
KANG, JH
TAKEICHI, N
KOBAYASHI, H
KOJIMA, Y
UNE, Y
UCHINO, J
机构：
[1] HOKKAIDO UNIV,GRAD SCH ENVIRONM SCI,DEPT ENVIRONM MED,SAPPORO,HOKKAIDO 060,JAPAN
[2] HOKKAIDO UNIV,SCH MED,DEPT SURG 1,SAPPORO,HOKKAIDO 060,JAPAN
来源：
JOURNAL OF CLINICAL INVESTIGATION | 1991年 / 87卷 / 05期
关键词：
FULMINANT HEPATIC FAILURE; TRACE METAL; COPPER TOXICITY; METABOLISM; GENETIC DISORDER;
D O I：
10.1172/JCI115208
中图分类号：
R-3 [医学研究方法]; R3 [基础医学];
学科分类号：
1001 ;
摘要：
Long-Evans Cinnamon (LEC) rats, an inbred strain of a mutant rat isolated from Long-Evans rats, develop hereditary hepatitis. To elucidate the role of copper metabolism in the development of the hepatitis in LEC rats, we examined the copper concentration in the tissues and serum levels of copper and ceruloplasmin. Copper concentration in the liver of LEC rats was over 40 times that of normal Long-Evans Agouti (LEA) rats, while the serum ceruloplasmin and copper concentrations in LEC rats decreased significantly. The hepatocytes of LEC rats show steatosis in cytoplasm and pleomorphism of mitochondria, resembling the histologic features of the liver in Wilson's disease. These findings suggest that the hereditary hepatitis in LEC rats is closely associated with copper toxicity, and may be dealing with a rat form of Wilson's disease. Thus the LEC rats will provide a unique and useful animal model for clarifying the mechanism and for developing treatment strategies for Wilson's disease and other abnormal copper metabolism in humans.
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页码：1858 / 1861
页数：4
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