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• W1783274641 abstract "Long-Evans Cinnamon rats develop a necrotizing hepatitis characterized by excessive hepatic copper accumulation, defective holoceruloplasmin biosynthesis and impaired biliary copper excretion. To elucidate the molecular basis of this defect, a cDNA clone encoding the rat Wilson disease gene was isolated and used to examine gene expression in selected tissues from normal and Long-Evans Cinnamon rats. Although this cDNA readily detects Wilson transcripts in liver and other tissues from normal rats, such transcripts are entirely absent from tissues derived from the Long-Evans Cinnamon rat strain. These data therefore identify the Long-Evans Cinnamon rat as the first bona fide animal model of Wilson disease and suggest that this rat strain may be a valuable resource in the study of this genetic disorder." @default.
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• W1783274641 date "1994-07-01" @default.
• W1783274641 modified "2023-10-15" @default.
• W1783274641 title "Expression of the Wilson disease gene is deficient in the Long-Evans Cinnamon rat" @default.
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• W1783274641 doi "https://doi.org/10.1042/bj3010001" @default.
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