Antioxidant defence systems and generation of reactive oxygen species in osteosarcoma cells with defective mitochondria: effect of selenium

Biochim Biophys Acta. Jun-Jul 2010;1797(6-7):890-6. doi: 10.1016/j.bbabio.2010.01.035. Epub 2010 Feb 4.


Mitochondrial diseases originate from mutations in mitochondrial or nuclear genes encoding for mitochondrial proteome. Neurogenic muscle weakness, ataxia and retinitis pigmentosa (NARP) syndrome is associated with the T8993G transversion in ATP6 gene which results in substitution at the very conservative site in the subunit 6 of mitochondrial ATP synthase. Defects in the mitochondrial respiratory chain and the ATPase are considered to be accompanied by changes in the generation of reactive oxygen species (ROS). This study aimed to elucidate effects of selenium on ROS and antioxidant system of NARP cybrid cells with 98% of T8993G mutation load. We found that selenium decreased ROS generation and increased the level and activity of antioxidant enzymes such as glutathione peroxidase (GPx) and thioredoxin reductase (TrxR). Therefore, we propose selenium to be a promising therapeutic agent not only in the case of NARP syndrome but also other diseases associated with mitochondrial dysfunctions and oxidative stress.

MeSH terms

  • Antioxidants / metabolism*
  • Antioxidants / pharmacology
  • Catalase / metabolism
  • Cell Line, Tumor
  • DNA, Mitochondrial / genetics
  • Humans
  • Hybrid Cells
  • Mitochondria / drug effects*
  • Mitochondria / genetics
  • Mitochondria / metabolism*
  • Mitochondrial Myopathies / drug therapy
  • Mitochondrial Myopathies / genetics
  • Mitochondrial Myopathies / metabolism
  • Mitochondrial Proton-Translocating ATPases / genetics
  • Mutation, Missense
  • NF-E2-Related Factor 2 / metabolism
  • Osteosarcoma / drug therapy*
  • Osteosarcoma / metabolism*
  • Reactive Oxygen Species / metabolism*
  • Retinitis Pigmentosa / drug therapy
  • Retinitis Pigmentosa / genetics
  • Retinitis Pigmentosa / metabolism
  • Selenium / pharmacology*
  • Superoxide Dismutase / metabolism
  • Syndrome
  • Thioredoxin-Disulfide Reductase / metabolism


  • Antioxidants
  • DNA, Mitochondrial
  • MT-ATP6 protein, human
  • NF-E2-Related Factor 2
  • NFE2L2 protein, human
  • Reactive Oxygen Species
  • Catalase
  • Superoxide Dismutase
  • Thioredoxin-Disulfide Reductase
  • Mitochondrial Proton-Translocating ATPases
  • Selenium

Supplementary concepts

  • Neuropathy ataxia and retinitis pigmentosa