Abstract
The redox status of mitochondrial coenzyme Q (CoQ) is an important marker for oxidative stress associated with several disorders such as Parkinson disease and Alzheimer disease. Altered redox status may be present in mitochondrial electron transport complex disorders. Intracellular CoQ levels reflect the functional status of the mitochondrial electron transport complex better than plasma levels. Here, we describe the method to determine the reduced and oxidized form of CoQ in white blood cells using LC–MS/MS.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
References
Bentinger, M., Brismar, K., and Dallner, G. (2007) The antioxidant role of coenzyme Q, Mitochondrion 7 Suppl, S41–50.
Haas, R. H., Parikh, S., Falk, M. J., Saneto, R. P., Wolf, N. I., Darin, N., Wong, L. J., Cohen, B. H., and Naviaux, R. K. (2008) The in-depth evaluation of suspected mitochondrial disease, Mol Genet Metab 94, 16–37.
Gotz, M. E., Gerstner, A., Harth, R., Dirr, A., Janetzky, B., Kuhn, W., Riederer, P., and Gerlach, M. (2000) Altered redox state of platelet coenzyme Q10 in Parkinson’s disease, J Neural Transm 107, 41–48.
Tang, P. H., Miles, M. V., Miles, L., Quinlan, J., Wong, B., Wenisch, A., and Bove, K. (2004) Measurement of reduced and oxidized coenzyme Q9 and coenzyme Q10 levels in mouse tissues by HPLC with coulometric detection, Clin Chim Acta 341, 173–184.
Vasta, V., Sedensky, M., Morgan, P., and Hahn, S. H. (2011) Altered redox status of coenzyme Q9 reflects mitochondrial electron transport chain deficiencies in Caenorhabditis elegans, Mitochondrion 11, 136–138.
Duncan, A. J., Heales, S. J., Mills, K., Eaton, S., Land, J. M., and Hargreaves, I. P. (2005) Determination of coenzyme Q10 status in blood mononuclear cells, skeletal muscle, and plasma by HPLC with di-propoxy-coenzyme Q10 as an internal standard, Clin Chem 51, 2380–2382.
Sacconi, S., Trevisson, E., Salviati, L., Ayme, S., Rigal, O., Redondo, A. G., Mancuso, M., Siciliano, G., Tonin, P., Angelini, C., Aure, K., Lombes, A., and Desnuelle, C. (2010) Coenzyme Q10 is frequently reduced in muscle of patients with mitochondrial myopathy, Neuromuscul Disord 20, 44–48.
Mollet, J., Giurgea, I., Schlemmer, D., Dallner, G., Chretien, D., Delahodde, A., Bacq, D., de Lonlay, P., Munnich, A., and Rotig, A. (2007) Prenyldiphosphate synthase, subunit 1 (PDSS1) and OH-benzoate polyprenyltransferase (COQ2) mutations in ubiquinone deficiency and oxidative phosphorylation disorders, J. Clin. Invest. 117:765–772.
Lopez, L. C., Schuelke, M., Quinzii, C. M., Kanki, T., Rodenburg, R.J., Naini, A., Dimauro, S., and Hirano, M. (2006) Leigh syndrome with nephropathy and CoQ10 deficiency due to decaprenyl diphosphate synthase subunit 2 (PDSS2) mutations. Am. J. Hum. Genet 79, 1125–1129.
Quizii, C., Niani, A., Salviati, L., Trevission, E., Navas, P., DiMauro, S., and Hirano, M (2006) A mutation in parahydroxybenoate-polyprenyltransferase (COQ2) mutations in ubiquinone deficiency and oxidative phosphorylation disorders. J. Clin. Invest. 117, 765–772.
Ruiz-Jimenez, J., Priego-Capote, F., Mata-Granados, J. M., Quesada, J. M., and Luque de Castro, M. D. (2007) Determination of the ubiquinol-10 and ubiquinone-10 (coenzyme Q10) in human serum by liquid chromatography tandem mass spectrometry to evaluate the oxidative stress, J Chromatogr A 1175, 242–248.
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2012 Springer Science+Business Media, LLC
About this protocol
Cite this protocol
Hahn, S.H., Kerfoot, S., Vasta, V. (2012). Assay to Measure Oxidized and Reduced Forms of CoQ by LC–MS/MS. In: Wong, Ph.D., LJ. (eds) Mitochondrial Disorders. Methods in Molecular Biology, vol 837. Humana Press. https://doi.org/10.1007/978-1-61779-504-6_11
Download citation
DOI: https://doi.org/10.1007/978-1-61779-504-6_11
Published:
Publisher Name: Humana Press
Print ISBN: 978-1-61779-503-9
Online ISBN: 978-1-61779-504-6
eBook Packages: Springer Protocols