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| Content Provider | World Health Organization (WHO)-Global Index Medicus |
|---|---|
| Author | Grefte, Sander Verkaart, Sjoerd Van Emst-de Vries, Sjenet E. Koopman, Werner J. H. Smeitink, Jan A. M. Willems, Peter H. G. M. Nijtmans, Leo G. J. |
| Description | Author Affiliation: Koopman WJ ( Department of Biochemistry, Nijmegen Centre for Molecular Life Sciences, Radboud University Nijmegen Medical Centre, The Netherlands. w.koopman@ncmls.ru.nl) |
| Abstract | Deficiency of mitochondrial NADH:ubiquinone oxidoreductase (complex I), is associated with a variety of clinical phenotypes such as Leigh syndrome, encephalomyopathy and cardiomyopathy. Circumstantial evidence suggests that increased reactive oxygen species (ROS) levels contribute to the pathogenesis of these disorders. Here we assessed the effect of the water-soluble vitamin E derivative Trolox on ROS levels, and the amount and activity of complex I in fibroblasts of six children with isolated complex I deficiency caused by a mutation in the NDUFS1 , NDUFS2, NDUFS7 , NDUFS8 or NDUFV1 gene. Patient cells displayed increased ROS levels and a variable decrease in complex I activity and amount. For control cells, the ratio between activity and amount was 1 whereas for the patients this ratio was below 1, indicating a defect in intrinsic catalytic activity of complex I in the latter cells. Trolox treatment dramatically reduced ROS levels in both control and patient cells, which was paralleled by a substantial increase in the amount of complex I. Although the ratio between the increase in activity and amount of complex I was exactly proportional in control cells it varied between 0.1 and 0.8 for the patients. Our findings suggest that the expression of complex I is regulated by ROS. Furthermore, they provide evidence that both the amount and intrinsic activity of complex I are decreased in inherited complex I deficiency. The finding that Trolox treatment increased the amount of complex I might aid the future development of antioxidant treatment strategies for patients. However, such treatment may only be beneficial to patients with a relatively small reduction in intrinsic catalytic defect of the complex. |
| ISSN | 00063002 |
| Journal | Biochimica et Biophysica Acta (BBA) - Reviews on Cancer |
| Issue Number | 7-8 |
| Volume Number | 1777 |
| Language | English |
| Publisher | Elsevier |
| Publisher Date | 2008-07-01 |
| Publisher Place | Netherlands |
| Access Restriction | Open |
| Subject Keyword | Chromans Pharmacology Electron Transport Complex I Deficiency Genetics Drug Effects Fibroblasts Enzymology Genetic Diseases, Inborn Kinetics Mitochondria Mutation Oxidative Phosphorylation Phenotype Protein Subunits Skin Research Support, Non-U.S. Gov't Biochemistry |
| Content Type | Text |
| Resource Type | Article |
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