Oxidative stress in inborn errors of metabolism: lessons from glutathione deficiency.
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.
Ristoff
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Karolinska Institutet, Children's Hospital, Huddinge University Hospital, Stockholm, Sweden.
Larsson
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A
D005721:Glutamate-Cysteine Ligase / Q000172:deficiency; D005978:Glutathione / Q000172:deficiency* / Q000378:metabolism; D005981:Glutathione Synthase / Q000172:deficiency; D006801:Humans; D008661:Metabolism, Inborn Errors / Q000378:metabolism*; D018384:Oxidative Stress
D005721:Glutamate-Cysteine Ligase; D005981:Glutathione Synthase; D005978:Glutathione