Skip to main content

Assay to Measure Oxidized and Reduced Forms of CoQ by LC–MS/MS

  • Protocol
  • First Online:
Book cover Mitochondrial Disorders

Part of the book series: Methods in Molecular Biology ((MIMB,volume 837))

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.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Protocol
USD 49.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 84.99
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 159.00
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 109.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Bentinger, M., Brismar, K., and Dallner, G. (2007) The antioxidant role of coenzyme Q, Mitochondrion 7 Suppl, S41–50.

    Article  PubMed  CAS  Google Scholar 

  2. 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.

    Article  PubMed  CAS  Google Scholar 

  3. 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.

    Article  PubMed  CAS  Google Scholar 

  4. 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.

    Article  PubMed  CAS  Google Scholar 

  5. 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.

    Article  PubMed  CAS  Google Scholar 

  6. 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.

    Article  PubMed  CAS  Google Scholar 

  7. 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.

    Article  PubMed  Google Scholar 

  8. 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.

    Google Scholar 

  9. 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.

    Article  PubMed  CAS  Google Scholar 

  10. 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.

    Google Scholar 

  11. 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.

    Article  PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Si Houn Hahn .

Editor information

Editors and Affiliations

Rights and permissions

Reprints 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

Publish with us

Policies and ethics