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J. Clin. Med. 2017, 6(4), 37; doi:10.3390/jcm6040037

The Value of Coenzyme Q10 Determination in Mitochondrial Patients

1
Clinical Biochemistry and Molecular Medicine Department, Institut de Recerca Sant Joan de Déu and CIBERER-ISCIII, Passeig Sant Joan de Déu, 2, 08950 Esplugues, Barcelona, Spain
2
Department of Blood Sciences, Royal Devon and Exeter NHS Foundation Trust, Exeter EX2 5DW, UK
*
Author to whom correspondence should be addressed.
Academic Editor: Iain P. Hargreaves
Received: 28 February 2017 / Revised: 17 March 2017 / Accepted: 17 March 2017 / Published: 24 March 2017
View Full-Text   |   Download PDF [818 KB, uploaded 24 March 2017]   |  

Abstract

Coenzyme Q10 (CoQ) is a lipid that is ubiquitously synthesized in tissues and has a key role in mitochondrial oxidative phosphorylation. Its biochemical determination provides insight into the CoQ status of tissues and may detect CoQ deficiency that can result from either an inherited primary deficiency of CoQ metabolism or may be secondary to different genetic and environmental conditions. Rapid identification of CoQ deficiency can also allow potentially beneficial treatment to be initiated as early as possible. CoQ may be measured in different specimens, including plasma, blood mononuclear cells, platelets, urine, muscle, and cultured skin fibroblasts. Blood and urinary CoQ also have good utility for CoQ treatment monitoring. View Full-Text
Keywords: coenzyme Q10 deficiency; mitochondrial diseases; treatment monitoring coenzyme Q10 deficiency; mitochondrial diseases; treatment monitoring
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Yubero, D.; Allen, G.; Artuch, R.; Montero, R. The Value of Coenzyme Q10 Determination in Mitochondrial Patients. J. Clin. Med. 2017, 6, 37.

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