Journal
FREE RADICAL BIOLOGY AND MEDICINE
Volume 165, Issue -, Pages 203-218Publisher
ELSEVIER SCIENCE INC
DOI: 10.1016/j.freeradbiomed.2021.01.017
Keywords
Coenzyme Q; Mitochondria; Neurological diseases; Secondary coenzyme Q deficiency; Brain; Muscle
Funding
- AFM-Telethon [23019]
- University of Angers
- CHU d'Angers
- Region Pays de la Loire
- Angers Loire Metropole
- Fondation Maladies Rares
- Association contre les Maladies Mitochondriales
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This article provides an overview of Coenzyme Q (CoQ) distribution in cells and tissues, secondary CoQ deficiency in neurological diseases, and therapeutic strategies for CoQ supplementation. It also discusses improvements in CoQ absorption, formulations, and imaging techniques.
Coenzyme Q (CoQ) is a ubiquitous lipid serving essential cellular functions. It is the only component of the mitochondrial respiratory chain that can be exogenously absorbed. Here, we provide an overview of current knowledge, controversies, and open questions about CoQ intracellular and tissue distribution, in particular in brain and skeletal muscle. We discuss human neurological diseases and mouse models associated with secondary CoQ deficiency in these tissues and highlight pharmacokinetic and anatomical challenges in exogenous CoQ biodistribution, recent improvements in CoQ formulations and imaging, as well as alternative therapeutical strategies to CoQ supplementation. The last section proposes possible mechanisms underlying secondary CoQ deficiency in human diseases with emphasis on neurological and neuromuscular disorders.
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