Chantale Langlois, Rossana Jorquera, Diana Orejuela, Anne Bergeron, Milton J. Finegold, William J. Rhead and Robert M. Tanguay,
Rescue from neonatal death in the murine model of hereditary tyrosinemia by glutathione monoethylester and vitamin C treatment,
Molecular Genetics and MetabolismIn Press, Corrected Proof, , Available online 26 November 2007.
In this article, the authors describe the administration of antioxidants to a mouse model of tyrosinemia type 1. The treatment prevented neonatal death, but the disease still progressed in the mice. Perhaps antioxidants could be used in the future in conjunction with NTBC, if further experiments in mice show that combination therapy decreases the incidence of hepatocellular carcinoma and hepatic dysplasia.
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Philippe Campeau, MD
Resident in Medical Genetics at McGill University
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