title: Targeting mitochondrial dysfunction and neurodegeneration by means of coenzyme Q10 and its analogues.
abstract: Coenzyme Q10 is a small electron carrier of the respiratory chain with antioxidant properties, widely used for the treatment of mitochondrial disorders. Mitochondrial diseases are neuromuscular disorders caused by impairment of the respiratory chain and increased generation of reactive oxygen species. Coenzyme Q10 supplementation is fundamental in patients with primary coenzyme Q10 deficiency. Furthermore, coenzyme Q10 and its analogues, idebenone and mitoquinone (or MitoQ), have been also used in the treatment of other neurogenetic/neurodegenerative disorders. In Friedreich ataxia idebenone may reduce cardiac hypertrophy and, at higher doses, also improve neurological function. These compounds may also play a potential role in other conditions which have been linked to mitochondrial dysfunction, such as Parkinson disease, Huntington disease, amyotrophic lateral sclerosis and Alzheimer disease. This review introduces mitochondrial disorders and Friedreich ataxia as two paradigms of the tight links existing between oxidative stress, respiratory chain dysfunction and neurodegeneration, and focuses on current and emerging therapeutic uses of coenzyme Q10 and idebenone in neurology.
authors.affiliation: Department of Neuroscience, Neurological Clinic, University of Pisa, Via Roma 67, 56126 Pisa, Italy. d.orsucci@sssup.it
authors.forename: D
authors.initials: D
authors.lastname: Orsucci
authors.forename: M
authors.initials: M
authors.lastname: Mancuso
authors.forename: E Caldarazzo
authors.initials: EC
authors.lastname: Ienco
authors.forename: A
authors.initials: A
authors.lastname: LoGerfo
authors.forename: G
authors.initials: G
authors.lastname: Siciliano
mesh_terms: D000818:Animals; D006801:Humans; D018977:Micronutrients / Q000378:metabolism / Q000494:pharmacology / Q000627:therapeutic use*; D008928:Mitochondria / Q000378:metabolism*; D028361:Mitochondrial Diseases / Q000188:drug therapy* / Q000378:metabolism / Q000503:physiopathology; D058990:Molecular Targeted Therapy; D019636:Neurodegenerative Diseases / Q000188:drug therapy* / Q000378:metabolism / Q000503:physiopathology; D014451:Ubiquinone / Q000031:analogs & derivatives* / Q000378:metabolism / Q000494:pharmacology / Q000502:physiology / Q000627:therapeutic use*
chemical_list: D018977:Micronutrients; D014451:Ubiquinone; C024989:coenzyme Q10