Article
Friedreich's ataxia: coenzyme Q10 and vitamin E therapy.
Mitochondrion - 1 Jun 2007
Cooper J M, Schapira A H V
Abstract excerpt
Since the identification of the genetic mutation causing Friedreich's ataxia (FRDA) our understanding of the mechanisms underlying disease pathogenesis have improved markedly. The genetic abnormality results in the deficiency of frataxin, a protein targeted to the mitochondrion. There is extensive evidence that mitochondrial respiratory chain dysfunction, oxidative damage and iron accumulation play significant...
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