AT long last: An animal model of Wilson's disease

Authors

  • Ronald J. Sokol M.D.

    1. Associate Professor of Pediatrics Pediatric Liver Center University of Colorado School of Medicine The Children's Hospital Denver, Colorado 80218
    Search for more papers by this author

Abstract

The LEC rat is an inbred mutant strain with spontaneous hepatitis isolated from Long-Evans rats. Since approximately 40% of LEC rats die of fulminant hepatitis, the rat serves an animal model for studying the pathogenesis and treatment of human fulminant hepatitis. The remaining 60% of LEC rats survive and develop chronic (prolonged) hepatitis and subsequently develop liver cancer. Therefore, the LEC rat serves an important animal model for studying the significance of chronic hepatitis in the development of human liver cancer, which often develops in association with chronic hepatitis. The LEC rat can also be used as an animal model of Wilson's disease, since recent studies have disclosed high copper accumulation in the liver and low ceruloplasmin concentration in the serum of this mutant rat.

Ancillary