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Erythrocyte glutathione peroxidase activity and plasma selenium concentration are reduced in maple syrup urine disease patients during treatment

Authors
Journal
International Journal of Developmental Neuroscience
0736-5748
Publisher
Elsevier
Publication Date
Volume
25
Issue
5
Identifiers
DOI: 10.1016/j.ijdevneu.2007.05.004
Keywords
  • Msud
  • Selenium
  • Glutathione Peroxidase
  • Oxidative Stress
Disciplines
  • Biology
  • Medicine

Abstract

Abstract Maple syrup urine disease (MSUD) is an inherited disorder caused by a deficiency of the branched-chain α-keto acid dehydrogenase complex activity. In the present study we evaluated selenium levels in plasma from MSUD patients at diagnosis and under treatment and the activities of glutathione peroxidase, catalase and superoxide dismutase in erythrocytes from treated patients. We verified that MSUD patients present a significant selenium deficiency at diagnosis, which becomes more pronounced during treatment, as well as a decrease of erythrocyte glutathione peroxidase activity during treatment. In contrast, erythrocyte catalase and superoxide dismutase activities were not altered in these patients. Our present results suggest that the reduction of an important antioxidant enzyme activity may be partially involved in the pathomechanisms of this disorder and that plasma selenium levels must be corrected through dietary supplementation in MSUD patients.

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