1. Analysis of reactive oxygen species and antioxidant defenses in complex I deficient patients revealed a specific increase in superoxide dismutase activity
- Author
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C. G. Suresh, Ashraf Yusuf Rangrez, Sharmila A. Bapat, Yogesh S. Shouche, Shama Barnabas, Himanshu Kumar, Milind S. Patole, and Aijaz Ahmed Wani
- Subjects
Male ,Mitochondrial Diseases ,Antioxidant ,Free Radicals ,medicine.medical_treatment ,Glutathione reductase ,medicine.disease_cause ,Biochemistry ,Antioxidants ,Superoxide dismutase ,chemistry.chemical_compound ,medicine ,Humans ,Lymphocytes ,RNA, Messenger ,Child ,chemistry.chemical_classification ,Reactive oxygen species ,Electron Transport Complex I ,biology ,Superoxide Dismutase ,Glutathione peroxidase ,Mitochondrial Myopathies ,General Medicine ,Glutathione ,Molecular biology ,Oxidative Stress ,chemistry ,Catalase ,biology.protein ,Female ,Reactive Oxygen Species ,Protein Processing, Post-Translational ,Oxidative stress - Abstract
The mechanism of free radical production by complex I deficiency is ill-defined, although it is of significant contemporary interest. This study studied the ROS production and antioxidant defenses in children with mitochondrial NADH dehydrogenase deficiency. ROS production has remained significantly elevated in patients compared to controls. The expression of all antioxidant enzymes significantly increased at mRNA level. However, the enzyme activities did not correlate with high mRNA or protein expression. Only the activity of superoxide dismutase (SOD) was found to correlate with higher mRNA expression in patient derived cell lines. The activities of the enzymes such as glutathione peroxidase (GPx), Catalase (CAT) and glutathione-S-transferase (GST) were significantly reduced in patients (p
- Published
- 2008
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