Cited by 92Coenzyme Q10 studies and effectsMitochondrial Function and PathologyMetabolism and Genetic Disorders

The evidence basis for coenzyme Q therapy in oxidative phosphorylation disease

Haas, Richard H.·2007·Mitochondrion

Abstract

The evidence supporting a treatment benefit for coenzyme Q10 (CoQ10) in primary mitochondrial disease (mitochondrial disease) whilst positive is limited. Mitochondrial disease in this context is defined as genetic disease causing an impairment in mitochondrial oxidative phosphorylation (OXPHOS). There are no treatment trials achieving the highest Level I evidence designation. Reasons for this include the relative rarity of mitochondrial disease, the heterogeneity of mitochondrial disease, the natural cofactor status and easy 'over the counter availability' of CoQ10 all of which make funding for the necessary large blinded clinical trials unlikely. At this time the best evidence for efficacy comes from controlled trials in common cardiovascular and neurodegenerative diseases with mitochondrial and OXPHOS dysfunction the etiology of which is most likely multifactorial with environmental factors playing on a background of genetic predisposition. There remain questions about dosing, bioavailability, tissue penetration and intracellular distribution of orally administered CoQ10, a compound which is endogenously produced within the mitochondria of all cells. In some mitochondrial diseases and other commoner disorders such as cardiac disease and Parkinson's disease low mitochondrial or tissue levels of CoQ10 have been demonstrated providing an obvious rationale for supplementation. This paper discusses the current state of the evidence supporting the use of CoQ10 in mitochondrial disease.

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Crossref · 10.1016/j.mito.2007.03.008

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Authors
Haas, R. H.
Affiliations
University of California San Diego (US)
Corresponding author
Haas, Richard H.
Venue
Mitochondrion
Publisher
Elsevier BV
Published
2007-06
Volume
7
Pages
S136-S145
ISSN
1567-7249 (ISSN-L)
OpenAlex
W2015731982
Cited by
92
References
75
Citations by year
2026: 1 · 2025: 3 · 2024: 4 · 2023: 5 · 2022: 9 · 2021: 5 · 2020: 4 · 2019: 1 · 2018: 5 · 2017: 2 · 2016: 4 · 2015: 2 · 2014: 6 · 2013: 5 · 2012: 8
FWCI
2.65
Type
journal article
Language
en
Open access
closed
Locations
  • Mitochondrion (publishedVersion)
  • PubMed (publishedVersion)
Funders
Wright Family Foundation
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 › Biochemistry, Genetics and Molecular Biology › Clinical Biochemistry › Metabolism and Genetic Disorders
Keywords
coenzyme Q10, mitochondrial disease, oxidative phosphorylation, primary mitochondrial disease, coenzyme Q10 supplementation, evidence base, clinical trial feasibility, Parkinson's disease, heart disease, neurodegenerative diseases
MeSH terms
Animals, Antioxidants, Cardiovascular Diseases, Coenzymes, Guinea Pigs, Humans, Mitochondria, Models, Biological, Oxidative Phosphorylation, Oxygen, Phosphorylation, Ubiquinone, Evidence-Based Medicine, Mitochondrial Diseases, Rats

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