Disabled early recruitment of antioxidant defenses in Friedreich's ataxia - PubMed (original) (raw)

Comparative Study

. 2001 Sep 15;10(19):2061-7.

doi: 10.1093/hmg/10.19.2061.

Affiliations

• PMID: 11590123
• DOI: 10.1093/hmg/10.19.2061

Comparative Study

Disabled early recruitment of antioxidant defenses in Friedreich's ataxia

K Chantrel-Groussard et al. Hum Mol Genet. 2001.

Abstract

Friedreich's ataxia (FRDA) results from a generalized deficiency of mitochondrial iron-sulfur protein activity ascribed to mitochondrial iron overload. However, iron overload appears to be a late event in the disease. Here we show that neither superoxide dismutases nor the import iron machinery was induced by an endogenous oxidative stress in FRDA patients' fibroblasts in contrast to control cells. Superoxide dismutase activity was not induced in the heart of conditional frataxin-KO mice either. This suggests that continuous oxidative damage to iron-sulfur clusters, resulting from hampered superoxide dismutase signaling, is causative of the mitochondrial deficiency and long term mitochondrial iron overload occurring in FRDA.

PubMed Disclaimer

Publication types

MeSH terms

Substances

LinkOut - more resources

• Full Text Sources

  • Ovid Technologies, Inc.
  • Silverchair Information Systems

• Other Literature Sources

  • The Lens - Patent Citations

• Medical

  • MedlinePlus Health Information

• Research Materials

  • NCI CPTC Antibody Characterization Program