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Oxidative stress in inborn errors of metabolism: Lessons from glutathione deficiency
Author(s) -
Ristoff E.,
Larsson A.
Publication year - 2002
Publication title -
journal of inherited metabolic disease
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.462
H-Index - 102
eISSN - 1573-2665
pISSN - 0141-8955
DOI - 10.1023/a:1015634032042
Subject(s) - oxidative stress , methylmalonic aciduria , inborn error of metabolism , glutathione , pathogenesis , metabolism , glutathione metabolism , biology , endocrinology , medicine , biochemistry , enzyme , methylmalonic acid , vitamin b12
Oxidative stress has been thought to be involved in the pathogenesis of several inborn errors of metabolism, such as mitochondrial enzyme deficiencies, G6PD deficiency, methylmalonic acidaemia and PKU. The possible role of oxidative stress in the pathogenesis of inborn errors of metabolism is discussed in the light of inborn errors in the metabolism of glutathione.