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In vitro coenzyme Q 10 treatment of forearm skin fibroblasts isolated from patients with SCA2 was found to reduce oxidative stress and normalize complex I and II-III activity of the mitochondrial respiratory chain (82)

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These observations strongly support the current hypothesis that reactive metabolite formation and protein binding especially in mitochondria are the key initiating events in the toxicity, which is then the trigger for a moderate mitochondrial oxidant stress with release of superoxide by complex III towards the cytosol without compromise of mitochondrial respiration [33]

This article is republished from The Conversation, a nonprofit, independent news organization bringing you facts and trustworthy analysis to help you make sense of our complex world

10.12688/f1000research.163124.1 21 FernndezM.BianchiM.Lux-LantosV.LibertunC