The Ubiquinone-Ubiquinol Redox Cycle and Its Clinical Consequences: An Overview.

Publisher: MDPI Country of Publication: Switzerland NLM ID: 101092791 Publication Model: Electronic Cited Medium: Internet ISSN: 1422-0067 (Electronic) Linking ISSN: 14220067 NLM ISO Abbreviation: Int J Mol Sci Subsets: MEDLINE

Original Publication: Basel, Switzerland : MDPI, [2000-

Ubiquinone*/analogs & derivatives ; Ubiquinone*/metabolism ; Ubiquinone*/deficiency ; Oxidation-Reduction*; Humans ; Mitochondria/metabolism ; Animals ; Selenium/metabolism ; Ataxia ; Muscle Weakness ; Mitochondrial Diseases

Coenzyme Q10 (CoQ10) plays a key role in many aspects of cellular metabolism. For CoQ10 to function normally, continual interconversion between its oxidised (ubiquinone) and reduced (ubiquinol) forms is required. Given the central importance of this ubiquinone-ubiquinol redox cycle, this article reviews what is currently known about this process and the implications for clinical practice. In mitochondria, ubiquinone is reduced to ubiquinol by Complex I or II, Complex III (the Q cycle) re-oxidises ubiquinol to ubiquinone, and extra-mitochondrial oxidoreductase enzymes participate in the ubiquinone-ubiquinol redox cycle. In clinical terms, the outcome of deficiencies in various components associated with the ubiquinone-ubiquinol redox cycle is reviewed, with a particular focus on the potential clinical benefits of CoQ10 and selenium co-supplementation.

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Keywords: Q cycle; coenzyme Q10; oxidoreductases; selenium; ubiquinol; ubiquinone

1339-63-5 (Ubiquinone)
M9NL0C577Y (ubiquinol)
EJ27X76M46 (coenzyme Q10)
H6241UJ22B (Selenium)

Coenzyme Q10 Deficiency

Date Created: 20240627 Date Completed: 20240627 Latest Revision: 20240629

20240629

PMC11203502

10.3390/ijms25126765

38928470