Dissolution of copper-rich granules in hepatic lysosomes by D-penicillamine prevents the development of fulminant hepatitis in Long-Evans cinnamon rats
D Klein, J Lichtmannegger, U Heinzmann… - Journal of …, 2000 - Elsevier
Background/Aim: The Long-Evans cinnamon rat has a mutation homologous to the human
Wilson disease gene, leading to gross copper accumulation and the development of
hepatitis. D-penicillamine, a copper-chelating drug widely and efficiently used in treating
Wilson disease, has also been shown to prevent hepatitis in Long-Evans cinnamon rats. The
objectives of this study were: i) to investigate the effectiveness of D-penicillamine when
administered to the already affected animals, and ii) to elucidate the mechanism of action of …
Wilson disease gene, leading to gross copper accumulation and the development of
hepatitis. D-penicillamine, a copper-chelating drug widely and efficiently used in treating
Wilson disease, has also been shown to prevent hepatitis in Long-Evans cinnamon rats. The
objectives of this study were: i) to investigate the effectiveness of D-penicillamine when
administered to the already affected animals, and ii) to elucidate the mechanism of action of …