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Coenzyme Q10 in the treatment of mitochondrial disease

Neergheen, V, Chalasani, A, Wainwright, L, Yubero, D, Montero, R, Artuch, R and Hargreaves, IP (2017) Coenzyme Q10 in the treatment of mitochondrial disease. Journal of Inborn Errors of Metabolism and Screening. ISSN 2326-4098

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Abstract

Currently, there is a paucity of available treatment strategies for oxidative phosphorylation disorders. Coenzyme Q10 (CoQ10) and related synthetic quinones are the only agents to date that have proven to be beneficial in the treatment of these heterogeneous disorders. The therapeutic efficacy of CoQ10 is not restricted to patients with an underlying CoQ10 deficiency and is thought to result from its ability to restore electron flow in the mitochondrial respiratory chain (MRC) as well as to increase the cellular antioxidant capacity. At present, however, there is no consensus on the appropriate dosage or therapeutic plasma level of CoQ10, and this information will be required before CoQ10 can be utilized effectively in the treatment of mitochondrial disease. The following review will outline our current knowledge on the use of CoQ10 in the treatment of MRC disorders and primary CoQ10 deficiencies. Keywords

Item Type: Article
Subjects: Q Science > QH Natural history > QH301 Biology
Q Science > QR Microbiology
Divisions: Pharmacy & Biomolecular Sciences
Publisher: Sage
Date Deposited: 01 Jun 2018 09:13
Last Modified: 01 Jun 2018 09:13
DOI or Identification number: 10.1177/2326409817707771
URI: http://researchonline.ljmu.ac.uk/id/eprint/8754

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