Article
Abnormal glutathione conjugation in patients with tyrosinaemia type I.
Journal of inherited metabolic disease - 1 Aug 1997
Mulders T M, Bergman D J, Poll-The B T, Smit G P, Breimer D D, Mulder G J, Duran M, Smeitink J A
Abstract excerpt
Previous studies have suggested that tyrosinaemia type I may be associated with reduced glutathione availability due to conjugation of tyrosinaemia-associated reactive intermediates with glutathione. In the present study, the glutathione/ glutathione S-transferase system of two tyrosinaemia patients and three healthy controls were characterized by administering the racemic sedative drug bromisoval, a probe drug...
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