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. (J. Clin. Invest. 1991. 87:1858-1861

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TiB 2 based hard material has been fabricated by combustion synthesis from elemental powders using Fe-Al intermetallics as the binder phase. Both the crystal orientation map and the pole figures by electron backscatter diffraction analysis showed that the TiB 2 particles have no preferential orientation. Preheating of the power compact is helpful to increase the relative density and to reduce the particle size of the synthesised product, which increases the hardness of the product. The highest hardness and relative density were achieved for a composition of TiB 2 -20 vol..-%FeAl.