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- W2051279372 endingPage "175" @default.
- W2051279372 startingPage "166" @default.
- W2051279372 abstract "Friedreich ataxia is the most common hereditary ataxia. The signs and symptoms of the disorder derive from decreased expression of the protein frataxin, which is involved in iron metabolism. Frataxin chaperones iron for iron-sulfur cluster biogenesis and detoxifies iron in the mitochondrial matrix. Decreased expression of frataxin is associated with impairments of iron-sulfur cluster biogenesis and heme synthesis, as well as with mitochondrial dysfunction and oxidative stress. Compounds currently in clinical trials are directed toward improving mitochondrial function and lessening oxidative stress. Iron chelators and compounds that increase frataxin expression are under evaluation. Further elucidation of frataxin's function should lead to additional therapeutic approaches." @default.
- W2051279372 created "2016-06-24" @default.
- W2051279372 creator A5084961278 @default.
- W2051279372 date "2006-09-01" @default.
- W2051279372 modified "2023-09-26" @default.
- W2051279372 title "Iron Dysregulation in Friedreich Ataxia" @default.
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