Li Y, Togashi Y, Sato S, Emoto T, Kang J H, Takeichi N, Kobayashi H, Kojima Y, Une Y, Uchino J
Laboratory of Pathology, Cancer Institute, Hokkaido University School of Medicine, Sapporo, Japan.
J Clin Invest. 1991 May;87(5):1858-61. doi: 10.1172/JCI115208.
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.
长-伊文斯肉桂色（LEC）大鼠是从长-伊文斯大鼠中分离出的一种突变大鼠的近交系，会发展为遗传性肝炎。为了阐明铜代谢在LEC大鼠肝炎发展中的作用，我们检测了组织中的铜浓度以及血清铜和铜蓝蛋白水平。LEC大鼠肝脏中的铜浓度是正常长-伊文斯刺豚鼠（LEA）大鼠的40多倍，而LEC大鼠血清中的铜蓝蛋白和铜浓度显著降低。LEC大鼠的肝细胞在细胞质中表现出脂肪变性和线粒体多形性，类似于威尔逊病肝脏的组织学特征。这些发现表明，LEC大鼠的遗传性肝炎与铜毒性密切相关，可能是威尔逊病的一种大鼠形式。因此，LEC大鼠将为阐明机制以及开发针对威尔逊病和人类其他异常铜代谢的治疗策略提供独特且有用的动物模型。
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