Journal Article10.1212/WNL.38.6.892
Clinical and biochemical correlations in mitochondrial myopathies treated with coenzyme Q10.
Nereo Bresolin,L. Bet,A. Binda,Maurizio Moggio,G. Comi,F. Nador,C. Ferrante,A. Carenzi,Guglielmo Scarlato +8 more
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TL;DR: Throughout the treatment most of the patients showed a progressive reduction of serum lactate and pyruvate levels following standard muscle exercise and generally improved neurologic functions.
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Abstract: We tested the efficacy of coenzyme Q10 (ubidecarenone, CoQ10) therapy in patients with Kearns-Sayre syndrome and other mitochondrial myopathies with chronic progressive external ophthalmoplegia (CPEO). We treated seven patients for 1 year with daily oral administration of 120 mg of CoQ10. Throughout the treatment most of our patients showed a progressive reduction of serum lactate and pyruvate levels following standard muscle exercise and generally improved neurologic functions. The ECG and echocardiogram showed no significant changes in our patients. None of our patients showed any improvement in ptosis and CPEO.
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Oxidative stress in ALS: key role in motor neuron injury and therapeutic target.
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TL;DR: There is substantial evidence implicating oxidative stress as a central mechanism by which motor neuron death occurs, along with how oxidative damage triggers or exacerbates other neurodegenerative processes, and the trials of a variety of antioxidants as potential therapies for ALS are reviewed.
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TL;DR: Oral administration of coenzyme Q10 markedly attenuated striatal lesions produced by systemic administration of 3-nitropropionic acid and significantly increased life span in a transgenic mouse model of familial amyotrophic lateral sclerosis, providing further evidence that coen enzyme Q10 can exert neuroprotective effects that might be useful in the treatment of neurodegenerative diseases.
Oxidative Stress in Huntington's Disease
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Oxidative phosphorylation diseases. Disorders of two genomes.
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