期刊
FEBS LETTERS
卷 509, 期 1, 页码 101-105出版社
WILEY
DOI: 10.1016/S0014-5793(01)03140-4
关键词
Friedreich ataxia; frataxin; manganese superoxide dismutase; iron; nuclear factor-kappa B; triplet-repeat disease
资金
- NINDS NIH HHS [NS34192] Funding Source: Medline
Iron-mediated oxidative stress has been implicated in the pathology of the neurodegenerative disease Friedreich ataxia (FRDA). Here, we show that normal upregulation of the stress defense protein manganese superoxide dismutase (MnSOD) fails to occur in FRDA fibroblasts exposed to iron. This impaired induction was observed at iron levels in which increased activation of the redox-sensitive factor NF-kappaB was absent. Furthermore, MnSOD induction could only be partially suppressed by antioxidants. We conclude that an NF-kappaB-independent pathway that may not require free radical signaling is responsible for the reduction of MnSOD induction. This impairment could constitute both a novel defense mechanism against iron-mediated oxidative stress in cells with mitochondrial iron overload and conversely, an alternative source of free radicals that could contribute to the disease pathology. (C) 2001 Published by Elsevier Science B.V. on behalf of the Federation of European Biochemical Societies.
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