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Research Article Free access | 10.1172/JCI115208

Spontaneous hepatic copper accumulation in Long-Evans Cinnamon rats with hereditary hepatitis. A model of Wilson's disease.

Y Li, Y Togashi, S Sato, T Emoto, J H Kang, N Takeichi, H Kobayashi, Y Kojima, Y Une, and J Uchino

Laboratory of Pathology, Cancer Institute, Hokkaido University School of Medicine, Sapporo, Japan.

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Laboratory of Pathology, Cancer Institute, Hokkaido University School of Medicine, Sapporo, Japan.

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Laboratory of Pathology, Cancer Institute, Hokkaido University School of Medicine, Sapporo, Japan.

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Laboratory of Pathology, Cancer Institute, Hokkaido University School of Medicine, Sapporo, Japan.

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Laboratory of Pathology, Cancer Institute, Hokkaido University School of Medicine, Sapporo, Japan.

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Laboratory of Pathology, Cancer Institute, Hokkaido University School of Medicine, Sapporo, Japan.

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Laboratory of Pathology, Cancer Institute, Hokkaido University School of Medicine, Sapporo, Japan.

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Laboratory of Pathology, Cancer Institute, Hokkaido University School of Medicine, Sapporo, Japan.

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Laboratory of Pathology, Cancer Institute, Hokkaido University School of Medicine, Sapporo, Japan.

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Laboratory of Pathology, Cancer Institute, Hokkaido University School of Medicine, Sapporo, Japan.

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Published May 1, 1991 - More info

Published in Volume 87, Issue 5 on May 1, 1991
J Clin Invest. 1991;87(5):1858–1861. https://doi.org/10.1172/JCI115208.
© 1991 The American Society for Clinical Investigation
Published May 1, 1991 - Version history
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Abstract

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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