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Biochemistry of Coenzyme Q10

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Antioxidants in Andrology

Abstract

Human coenzyme Q (CoQ10) or ubiquinone is mainly known for its bioenergetic role as a proton and electron carrier in the inner mitochondrial membrane and is also an endogenous lipophilic antioxidant, ubiquitous in biological membranes. It is also present in plasma lipoproteins, where it plays a well-recognized antioxidant role. More recently coenzyme Q10 was also shown to affect gene expression by modulating the intracellular redox status. Its involvement in many cellular and extracellular functions suggests that its use as a food supplement could be beneficial in conditions associated with increased oxidative stress underlying different pathological conditions. In reproductive biology, CoQ10 has been shown to play a role in fertility of both males and females.

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References

  1. Balercia G, Buldreghini E, Vignini A, Tiano L, Paggi F, Amoroso S, Ricciardo-Lamonica G, Boscaro M, Lenzi A, Littarru G. Coenzyme Q10 treatment in infertile men with idiopathic asthenozoospermia: a placebo-controlled, double-blind randomized trial. Fertil Steril. 2009;91(5):1785–92. doi:10.1016/j.fertnstert.2008.02.119.

    Article  CAS  PubMed  Google Scholar 

  2. Belardinelli R, Muçaj A, Lacalaprice F, Solenghi M, Seddaiu G, Principi F, Tiano L, Littarru GP. Coenzyme Q10 and exercise training in chronic heart failure. Eur Heart J. 2006;27(22):2675–81.

    Article  CAS  PubMed  Google Scholar 

  3. Bentinger M, Tekle M, Brismar K, Chojnacki T, Swiezewska E, Dallner G. Polyisoprenoid epoxides stimulate the biosynthesis of coenzyme Q and inhibit cholesterol synthesis. J Biol Chem. 2008a;283(21):14645–53. doi:10.1074/jbc.M710202200.

    Article  CAS  PubMed  Google Scholar 

  4. Bentinger M, Tekle M, Brismar K, Chojnacki T, Swiezewska E, Dallner G. Stimulation of coenzyme Q synthesis. Biofactors. 2008b;32(1–4):99–111.

    Article  CAS  PubMed  Google Scholar 

  5. Bentov Y, Casper RF. The aging oocyte--can mitochondrial function be improved? Fertil Steril. 2013;99(1):18–22. doi:10.1016/j.fertnstert.2012.11.031apr.

    Article  CAS  PubMed  Google Scholar 

  6. Caso G, Kelly P, McNurlan MA, Lawson WE. Effect of coenzyme q10 on myopathic symptoms in patients treated with statins. Am J Cardiol. 2007;99(10):1409–12.

    Article  CAS  PubMed  Google Scholar 

  7. Crane FL, Hatefi Y, Lester RL, Widmer C. Isolation of a quinone from beef heart mitochondria. Biochim Biophys Acta. 1957;25(1):220–1.

    Article  CAS  PubMed  Google Scholar 

  8. Dallner G, Stocker R. Encyclopedia of dietary supplements. New York: Marcel Dekker; 2005. p. 121.

    Google Scholar 

  9. De Cabo R, Cabello R, Rios M, López-Lluch G, Ingram DK, Lane MA, Navas P. Calorie restriction attenuates age-related alterations in the plasma membrane antioxidant system in rat liver. Exp Gerontol. 2004;39(3):297–304.

    Article  CAS  PubMed  Google Scholar 

  10. Ernster L, Forsmark-Andrée P. Ubiquinol: an endogenous antioxidant in aerobic organisms. Clin Investig. 1993;71(8 Suppl):S60–5. PubMed PMID: 8241707.

    CAS  PubMed  Google Scholar 

  11. Estornell E, Fato R, Castelluccio C, Cavazzoni M, Parenti Castelli G, Lenaz G. Saturation kinetics of coenzyme Q in NADH and succinate oxidation in beef heart mitochondria. FEBS Lett. 1992;311(2):107–9. PubMed PMID: 1327877.

    Article  CAS  PubMed  Google Scholar 

  12. Genova ML, Bianchi C, Lenaz G. Supercomplex organization of the mitochondrial respiratory chain and the role of the Coenzyme Q pool: pathophysiological implications. Biofactors. 2005;25(1–4):5–20.

    Article  CAS  PubMed  Google Scholar 

  13. Green DE, Tzagoloff A. The mitochondrial electron transfer chain. Arch Biochem Biophys. 1966;116(1):293–304. PubMed PMID: 4289862.

    Article  CAS  PubMed  Google Scholar 

  14. Groneberg DA, Kindermann B, Althammer M, Klapper M, Vormann J, Littarru GP, Döring F. Coenzyme Q10 affects expression of genes involved in cell signalling, metabolism and transport in human CaCo-2 cells. Int J Biochem Cell Biol. 2005;37(6):1208–18. 9

    Article  CAS  PubMed  Google Scholar 

  15. Heida KY, Bots ML, de Groot CJ, van Dunné FM, Hammoud NM, Hoek A, Laven JS, Maas AH, Roeters van Lennep JE, Velthuis BK, Franx A. Cardiovascular risk management after reproductive and pregnancy-related disorders: a Dutch multidisciplinary evidence-based guideline. Eur J Prev Cardiol. 2016. pii: 2047487316659573. [Epub ahead of print] Review.

    Google Scholar 

  16. Kagan V, Serbinova E, Packer L. Antioxidant effects of ubiquinones in microsomes and mitochondria are mediated by tocopherol recycling. Biochem Biophys Res Commun. 1990;169(3):851–7. PubMed PMID: 2114108.

    Article  CAS  PubMed  Google Scholar 

  17. Kamei M, Fujita T, Kanbe T, Sasaki K, Oshiba K, Otani S, Matsui-Yuasa I, Morisawa S. The distribution and content of ubiquinone in foods. Int J Vitam Nutr Res. 1986;56(1):57–63. PubMed PMID: 3710719.

    CAS  PubMed  Google Scholar 

  18. Kröger A, Klingenberg M. Eur J Biochem. 1973;34:358.

    Article  PubMed  Google Scholar 

  19. Laaksonen R, Jokelainen K, Sahi T, Tikkanen MJ, Himberg JJ. Decreases in serum ubiquinone concentrations do not result in reduced levels in muscle tissue during short-term simvastatin treatment in humans. Clin Pharmacol Ther. 1995;57(1):62–6. PubMed PMID: 7828383.

    Article  CAS  PubMed  Google Scholar 

  20. Lamperti C, Naini AB, Lucchini V, Prelle A, Bresolin N, Moggio M, Sciacco M, Kaufmann P, DiMauro S. Muscle coenzyme Q10 level in statin-related myopathy. Arch Neurol. 2005;62(11):1709–12.

    Article  PubMed  Google Scholar 

  21. Littarru GP, Tiano L, Belardinelli R, Watts GF. Coenzyme Q(10), endothelial function, and cardiovascular disease. Biofactors. 2011;37(5):366–73. doi:10.1002/biof.154.

    Article  CAS  PubMed  Google Scholar 

  22. Littarru GP, Langsjoen P. Coenzyme Q10 and statins: biochemical and clinical implications. Mitochondrion. 2007;7 Suppl:S168–74. 7

    Article  PubMed  Google Scholar 

  23. Mancini A, De Marinis L, Littarru GP, Balercia G. An update of Coenzyme Q10 implications in male infertility: biochemical and therapeutic aspects. Biofactors. 2005;25(1–4):165–74.

    Article  CAS  PubMed  Google Scholar 

  24. Marcoff L, Thompson PD. The role of coenzyme Q10 in statin-associated myopathy: a systematic review. J Am Coll Cardiol. 2007;12;49(23):2231–7. Review. PubMed PMID: 17560286.

    Google Scholar 

  25. Mohr D, Bowry VW, Stocker R. Dietary supplementation with coenzyme Q10 results in increased levels of ubiquinol-10 within circulating lipoproteins and increased resistance of human low-density lipoprotein to the initiation of lipid peroxidation. Biochim Biophys Acta. 1992;1126(3):247–54. PubMed PMID: 1637852.

    Article  CAS  PubMed  Google Scholar 

  26. Montini G, Malaventura C, Salviati L. Early coenzyme Q10 supplementation in primary coenzyme Q10 deficiency. N Engl J Med. 2008;358(26):2849–50. doi:10.1056/NEJMc0800582.

    Article  CAS  PubMed  Google Scholar 

  27. Mordente A, Martorana GE, Santini SA, Miggiano GA, Petitti T, Giardina B, Battino M, Littarru GP. Antioxidant effect of coenzyme Q on hydrogen peroxide-activated myoglobin. Clin Investig. 1993;71(8 Suppl):S92–6. PubMed PMID: 8241712.

    CAS  PubMed  Google Scholar 

  28. Mortensen SA, Rosenfeldt F, Kumar A, Dolliner P, Filipiak KJ, Pella D, Alehagen U, Steurer G, GP L, Q-SYMBIO Study Investigators. The effect of coenzyme Q10 on morbidity and mortality in chronic heart failure: results from Q-SYMBIO: a randomized double-blind trial. JACC Heart Fail. 2014;2(6):641–9. doi:10.1016/j.jchf.2014.06.008.

    Article  PubMed  Google Scholar 

  29. Morton RA. Ubiquinone Nature. 1958;182(4652):1764–7.

    Article  CAS  PubMed  Google Scholar 

  30. Musicki B, Bella AJ, Bivalacqua TJ, Davies KP, DiSanto ME, Gonzalez-Cadavid NF, Hannan JL, Kim NN, Podlasek CA, Wingard CJ, Burnett AL. Basic science evidence for the link between erectile dysfunction and cardiometabolic dysfunction. J Sex Med. 2015;12(12):2233–55. doi:10.1111/jsm.13069. PubMed PMID: 26646025; PubMed Central PMCID: PMC4854187.

    Article  CAS  PubMed  Google Scholar 

  31. Naini A, Lewis VJ, Hirano M, DiMauro S. Primary coenzyme Q10 deficiency and the brain. Biofactors. 2003;18(1–4):145–52.

    Article  CAS  PubMed  Google Scholar 

  32. Navas P, Villalba JM, de Cabo R. The importance of plasma membrane coenzyme Q in aging and stress responses. Mitochondrion. 2007;7(Suppl):S34–40.

    Article  CAS  PubMed  Google Scholar 

  33. Pagano G, Aiello Talamanca A, Castello G, Cordero MD, d'Ischia M, Gadaleta MN, Pallardó FV, Petrović S, Tiano L, Zatterale A. Current experience in testing mitochondrial nutrients in disorders featuring oxidative stress and mitochondrial dysfunction: rational design of chemoprevention trials. Int J Mol Sci. 2014a;15(11):20169–208. doi:10.3390/ijms151120169. PubMed PMID: 25380523; PubMed Central PMCID: PMC4264162.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  34. Pagano G, Talamanca AA, Castello G, Cordero MD, d'Ischia M, Gadaleta MN, Pallardó FV, Petrović S, Tiano L, Zatterale A. Oxidative stress and mitochondrial dysfunction across broad-ranging pathologies: toward mitochondria-targeted clinical strategies. Oxidative Med Cell Longev. 2014b;2014:541230. doi:10.1155/2014/541230. PubMed PMID: 24876913; PubMed Central PMCID: PMC4024404.

    Article  Google Scholar 

  35. Perry JR, Murray A, Day FR, Ong KK. Molecular insights into the aetiology of female reproductive ageing. Nat Rev Endocrinol. 2015;11(12):725–34. doi:10.1038/nrendo.2015.167.

    Article  CAS  PubMed  Google Scholar 

  36. Phillipson OT. Management of the aging risk factor for Parkinson’s disease. Neurobiol Aging. 2014;35(4):847–57. doi:10.1016/j.neurobiolaging.2013.10.073.

    Article  PubMed  Google Scholar 

  37. Päivä H, Thelen KM, Van Coster R, Smet J, De Paepe B, Mattila KM, Laakso J, Lehtimäki T, von Bergmann K, Lütjohann D, Laaksonen R. High-dose statins and skeletal muscle metabolism in humans: a randomized, controlled trial. Clin Pharmacol Ther. 2005;78(1):60–8.

    Article  PubMed  Google Scholar 

  38. Quinzii CM, López LC, Von-Moltke J, Naini A, Krishna S, Schuelke M, Salviati L, Navas P, DiMauro S, Hirano M. Respiratory chain dysfunction and oxidative stress correlate with severity of primary CoQ10 deficiency. FASEB J. 2008b;22(6):1874–85. doi:10.1096/fj.07-100149. PubMed PMID: 18230681; PubMed Central PMCID: PMC2731482.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  39. Quinzii CM, López LC, Naini A, DiMauro S, Hirano M. Human CoQ10 deficiencies. Biofactors. 2008a;32(1–4):113–8. PubMed PMID: 19096106; PubMed Central PMCID: PMC3625975.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  40. Roberts JM, Mascalzoni D, Ness RB, Poston L, Global Pregnancy Collaboration. Collaboration to understand complex diseases: preeclampsia and adverse pregnancy outcomes. Hypertension. 2016;67(4):681–7. doi:10.1161/HYPERTENSIONAHA.115.06133.

    Article  CAS  PubMed  Google Scholar 

  41. Safarinejad MR. Efficacy of coenzyme Q10 on semen parameters, sperm function and reproductive hormones in infertile men. J Urol. 2009;182(1):237–48. doi:10.1016/j.juro.2009.02.121.

    Article  CAS  PubMed  Google Scholar 

  42. Salviati L, Sacconi S, Murer L, Zacchello G, Franceschini L, Laverda AM, Basso G, Quinzii C, Angelini C, Hirano M, Naini AB, Navas P, DiMauro S, Montini G. Infantile encephalomyopathy and nephropathy with CoQ10 deficiency: a CoQ10-responsive condition. Neurology. 2005;65(4):606–8.

    Article  CAS  PubMed  Google Scholar 

  43. Singh RB, Shinde SN, Chopra RK, Niaz MA, Thakur AS, Onouchi Z. Effect of coenzyme Q10 on experimental atherosclerosis and chemical composition and quality of atheroma in rabbits. Atherosclerosis. 2000;148(2):275–82.

    Article  CAS  PubMed  Google Scholar 

  44. Stojkovic M, Westesen K, Zakhartchenko V, Stojkovic P, Boxhammer K, Wolf E. Coenzyme Q(10) in submicron-sized dispersion improves development, hatching, cell proliferation, and adenosine triphosphate content of in vitro-produced bovine embryos. Biol Reprod. 1999;61(2):541–7.

    Article  CAS  PubMed  Google Scholar 

  45. Suzuki T, Nozawa T, Sobajima M, Igarashi N, Matsuki A, Fujii N, Inoue H. Atorvastatin-induced changes in plasma coenzyme q10 and brain natriuretic peptide in patients with coronary artery disease. Int Heart J. 2008;49(4):423–33.

    Article  CAS  PubMed  Google Scholar 

  46. Teran E, Hernandez I, Nieto B, Tavara R, Ocampo JE, Calle A. Coenzyme Q10 supplementation during pregnancy reduces the risk of pre-eclampsia. Int J Gynaecol Obstet. 2009;105(1):43–5. doi:10.1016/j.ijgo.2008.11.033.

    Article  CAS  PubMed  Google Scholar 

  47. Teran E, Escudero C, Vivero S, Molina G, Calle A. NO in early pregnancy and development of preeclampsia. Hypertension. 2006;47(4):e17. 3

    Article  CAS  PubMed  Google Scholar 

  48. Thomas SR, Witting PK, Stocker R. A role for reduced coenzyme Q in atherosclerosis? Biofactors. 1999;9(2–4):207–24.

    Article  CAS  PubMed  Google Scholar 

  49. Tiano L, Belardinelli R, Carnevali P, Principi F, Seddaiu G, Littarru GP. Effect of coenzyme Q10 administration on endothelial function and extracellular superoxide dismutase in patients with ischaemic heart disease: a double-blind, randomized controlled study. Eur Heart J. 2007;28(18):2249–55. PubMed PMID:17644511.

    Google Scholar 

  50. Tiano L, Busciglio J. Mitochondrial dysfunction and Down’s syndrome: is there a role for coenzyme Q(10) ? Biofactors. 2011;37(5):386–92. doi:10.1002/biof.184.

  51. Tirabassi G, Vignini A, Tiano L, Buldreghini E, Brugè F, Silvestri S, Orlando P, D'Aniello A, Mazzanti L, Lenzi A, Balercia G. Protective effects of coenzyme Q10 and aspartic acid on oxidative stress and DNA damage in subjects affected by idiopathic asthenozoospermia. Endocrine. 2015;49(2):549–52. doi:10.1007/s12020-014-0432-6.

    Article  CAS  PubMed  Google Scholar 

  52. Tomasetti M, Alleva R, Solenghi MD, Littarru GP. Distribution of antioxidants among blood components and lipoproteins: significance of lipids/CoQ10 ratio as a possible marker of increased risk for atherosclerosis. Biofactors. 1999;9(2–4):231–40.

    Article  CAS  PubMed  Google Scholar 

  53. Turi A, Giannubilo SR, Brugè F, Principi F, Battistoni S, Santoni F, Tranquilli AL, Littarru G, Tiano L. Coenzyme Q10 content in follicular fluid and its relationship with oocyte fertilization and embryo grading. Arch Gynecol Obstet. 2012;285(4):1173–6. doi:10.1007/s00404-011-2169-2.

    Article  CAS  PubMed  Google Scholar 

  54. Watts GF, Playford DA, Croft KD, Ward NC, Mori TA, Burke V. Coenzyme Q(10) improves endothelial dysfunction of the brachial artery in Type II diabetes mellitus. Diabetologia. 2002;45(3):420–6.

    Article  CAS  PubMed  Google Scholar 

  55. Willis R, Anthony M, Sun L, Honse Y, Qiao G. Clinical implications of the correlation between coenzyme Q10 and vitamin B6 status. Biofactors. 1999;9(2–4):359–63.

    Article  CAS  PubMed  Google Scholar 

  56. Witting PK, Pettersson K, Letters J, Stocker R. Anti-atherogenic effect of coenzyme Q10 in apolipoprotein E gene knockout mice. Free Radic Biol Med. 2000;29(3–4):295–305.

    Article  CAS  PubMed  Google Scholar 

  57. Young JM, Florkowski CM, Molyneux SL, McEwan RG, Frampton CM, George PM, Scott RS. Effect of coenzyme Q(10) supplementation on simvastatin-induced myalgia. Am J Cardiol. 2007;100(9):1400–3.

    Google Scholar 

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Littarru, G.P., Bruge, F., Tiano, L. (2017). Biochemistry of Coenzyme Q10. In: Balercia, G., Gandini, L., Lenzi, A., Lombardo, F. (eds) Antioxidants in Andrology. Trends in Andrology and Sexual Medicine. Springer, Cham. https://doi.org/10.1007/978-3-319-41749-3_2

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