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.
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MitoQ has been tested successfully in human diseases such as Hepatitis C induced liver disease and skin photo damage and in experimental animal models including ischemia-reperfusion, neurodegenerative diseases, diabetes, and alcohol-induced hepatosteatosis.
“For example, Mitoquinone (MitoQ) has been tested successfully in human diseases such as Hepatitis C induced liver disease and skin photo damage as well as a number of experimental animal models including ischemia-reperfusion, neurodegenerative diseases, diabetes, and alcohol-induced hepatosteatosis [8–14].”
From Mitochondrially targeted compounds and their impact on cellular bioenergetics (2013) · cites this paper as [12]
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- Authors
- Orsucci, D., Mancuso, M., Ienco, E. C., LoGerfo, A., & Siciliano, G.
- Affiliations
- University of Pisa (IT)
- Corresponding author
- Siciliano, G.
- Venue
- Current Medicinal Chemistry
- Publisher
- Bentham Science Publishers Ltd.
- Published
- 2011-09-01
- Volume
- 18
- Issue
- 26
- Pages
- 4053-4064
- ISSN
- 0929-8673 (ISSN-L)
- OpenAlex
- W2105461258
- Cited by
- 97
- References
- 0
- Citations by year
- 2026: 2 · 2025: 9 · 2024: 6 · 2023: 11 · 2022: 3 · 2021: 9 · 2020: 10 · 2019: 2 · 2018: 3 · 2017: 4 · 2016: 5 · 2015: 3 · 2014: 9 · 2013: 11 · 2012: 10
- FWCI
- 4.02
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Current Medicinal Chemistry (publishedVersion)
- PubMed (publishedVersion)
- CINECA IRIS Institutial research information system (University of Pisa) (submittedVersion)
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Coenzyme Q10 studies and effects
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Mitochondrial Function and Pathology
- Life Sciences › Neuroscience › Neurology › Neurological diseases and metabolism
- Keywords
- coenzyme Q10, idebenone, mitoquinone, mitochondrial dysfunction, neurodegeneration, Friedreich's ataxia, mitochondrial disease, electron transport chain, oxidative stress, reactive oxygen species, Parkinson's disease, Huntington's disease, amyotrophic lateral sclerosis, Alzheimer's disease, coenzyme Q10 deficiency, antioxidant therapy
- MeSH terms
- Animals, Humans, Mitochondria, Ubiquinone, Micronutrients, Neurodegenerative Diseases, Mitochondrial Diseases, Molecular Targeted Therapy
About these checks
Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.
Statistics and arithmetic are recomputed, not judged.
Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.
What counts as a claim ›How we measure confidence ›How we check this ›
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