Coenzyme Q

high-resolution terminology - matching measurements at high-resolution

Coenzyme Q

Description

Coenzyme Q or ubiquinone (2,3-dimethoxy-5-methyl-6-polyprenyl-1,4-benzoquinone) was discovered in 1957 by the group of Crane. It is a lipid composed of a benzoquinone ring with an isoprenoid side chain, two methoxy groups and one methyl group. The length of the isoprenoid chain varies depending on the species; for example, six isoprenoid units (CoQ₆) is the most commonly found CoQ in Saccharomyces cerevisiae, eight units in Escherichia coli (CoQ₈), nine units in Caenorhabditis elegans and rodents (CoQ₉), ten units in humans (CoQ₁₀), and some species have more than one CoQ form, e.g. human and rodent mitochondria contain different proportions of CoQ₉ and CoQ₁₀. These redox compounds exist in three different forms: quinone (oxidized), quinol (reduced), and an intermediate semiquinone.

More details » Q-junction

Abbreviation: Q, CoQ

Reference: Aberg 1992 Arch Biochem Biophys, Aussel 2014 Biochim Biophys Acta, Awad 2018 Essays Biochem, Crane 1956 Biochim Biophys Acta, Gnaiger 2020 BEC MitoPathways, Hernández-Camacho 2018 Frontiers in physiology, Song 2011 Free Radical Biol Med, Watts 2017 Genetics, Wolf 1958 J Am Chem Soc

Nomenclature

Different forms are used to write coenzyme Q-10 in the literature: CoQ₁₀ or CoQ10 or CoQ-10.

CoQ₁₀ is most commonly used in biochemistry, in line with EMBL (ChEBI ontology): https://www.ebi.ac.uk/chebi/chebiOntology.do?chebiId=CHEBI:46245. Accordingly, the number N of isoprenoid units is indicated as CoQ_N.
Q-n is recommended by IUPAC for ubiquinone-n: https://iupac.qmul.ac.uk/misc/quinone.html

CoQ₁₀ refers to all protonation states and the three redox states. It is useful to specify redox states by distinguishing CoQ₁₀ (all species) from the oxidized ubiquinone-10 (UQ₁₀), the reduced ubiquinol-10 (UQ₁₀H₂), and the free radical ubisemiquinone (USQ₁₀^•−). Song and Buettner (2011) use Q/SQ^•−/H₂Q for the quinone/semiquinone/hydroquinone triad. Whereas H₂Q fits the term hydroquinone, the most commonly used term is (ubi)quinol with the corresponding acronyms QH₂ and UQH₂.

The redox states of plant plastoquinone-9 can be distinguished as PQ₉ and PQ₉H₂. Hauvaux (2020) uses PQ-9 for the total pool of plastoquinone-9.

Coenzyme Q
Name	State	Abbreviation	Example
coenzyme Q	all protonation and redox states	CoQ	CoQ₁₀
ubiquinone	oxidized	UQ	UQ₁₀
ubiquinol	reduced	UQH₂	UQ₁₀H₂
ubisemiquinone	free radical	USQ^•−	USQ₁₀^•−

Application in HRR

See: Coenzyme Q2

Keywords

Bioblast links: Q - >>>>>>> - Click on [Expand] or [Collapse] - >>>>>>>

Coenzyme Q

» Coenzyme Q

» Quinone, Ubiquinone Q; oxidized

» Quinol, Ubiquinol QH₂; reduced

» Semiquinone

» Coenzyme Q2

» Q-redox state

» Q-pools

Mitochondrial pathways, respiratory Complexes, and Q

» Q-cycle

» Q-junction

» Convergent electron flow

» NS-pathway

» FNS

» FNSGp

» N-pathway

» Reverse electron flow from CII to CI

» CI

» Rotenone

» Amytal

» Piericidin

» S-pathway

» CII

» Malonate

» F-pathway

» CETF, Electron-transferring flavoprotein complex

» Gp-pathway

» CGpDH, Glycerophosphate dehydrogenase complex

» CIII

» Myxothiazol

» Choline dehydrogenase

» Dihydro-orotate dehydrogenase

NextGen-O2k and Q-Module

» NextGen-O2k

» Q-Module

» Q-Sensor

» Cyclic voltammetry

» Three-electrode system

General

» Categories of SUIT protocols

» Electron transfer pathway

» Electron-transfer-pathway state

» F-junction

» N-junction

MitoPedia: mitObesity drugs

- >>>>>>> - Click on [Collapse] or [Expand] - >>>>>>>

Term	Abbreviation	Description
Aspirin		Aspirin is a widely applied drug that requires dosage adjusted to individual body mass. It is a non-selective COX inhibitor and exerts an effect on long-chain fatty acid transport into mitochondria.
Curcumin		Curcumin has been shown to possess significant anti-inflammatory, anti-oxidant, anti-carcinogenic, anti-mutagenic, anti-coagulant and anti-infective effects. The protective effects of curcumin on rat heart mitochondrial injuries induced by in vitro anoxia–reoxygenation were evaluated by Xu et al 2013. It was found that curcumin added before anoxia or immediately prior to reoxygenation exhibited remarkable protective effects against anoxia–reoxygenation induced oxidative damage to mitochondria.
Elamipretide	Bendavia	Bendavia (Elamipretide) was developed as a mitochondria-targeted drug against degenerative diseases, including cardiac ischemia-reperfusion injury. Clinical trials showed variable results. It is a cationic tetrapeptide which readily passes cell membranes, associates with cardiolipin in the mitochondrial inner membrane. Supercomplex-associated CIV activity significantly improved in response to elamipretide treatment in the failing human heart.
Flavonoids		Flavonoids are a group of bioactive polyphenols with potential antioxidant and anti-inflammatory effects, abundant in fruits and vegetables, and in some medicinal herbs. Flavonoids are synthesized in plants from phenylalanine. Dietary intake of flavonoids as nutraceuticals is discussed for targeting T2D and other degenerative diseases.
Melatonin	aMT	Melatonin (N-acetyl-5-methoxytryptamine, aMT) is a highly conserved molecule present in unicellular to vertebrate organisms. Melatonin is synthesized from tryptophan in the pinealocytes by the pineal gland and also is produced in other organs, tissues and fluids (extrapineal melatonin). Melatonin has lipophilic and hydrophilic nature which allows it to cross biological membranes. Therefore, melatonin is present in all subcellular compartments predominantly in the nucleus and mitochondria. Melatonin has pleiotropic functions with powerful antioxidant, anti-inflammatory and oncostatic effects with a wide spectrum of action particularly at the level of mitochondria. » MiPNet article
Metformin		Metformin is mainly known as an important antidiabetic drug which is effective, however, in a wide spectrum of degenerative diseases. It is an inhibitor of Complex I and glycerophosphate dehydrogenase complex.
Rapamycin		Rapamycin is an inhibitor of the mammalian/mechanistic target of rapamycin, complex 1 (mTORC1). Rapamycin induces autophagy and dyscouples mitochondrial respiration. Rapamycin delays senescence in human cells, and extends lifespan in mice without detrimental effects on mitochondrial fitness in skeletal muscle.
Resveratrol		Resveratrol is a natural bioactive phenol prouced by several plants with antioxidant and anti-inflammatory effects. Dietary intake as nutraceutical is discussed for targeting mitochondria with a wide spectrum of action in degenerative diseases.
Spermidine		Spermidine is a polycationic bioactive polyamine mainly found in wheat germ, soybean and various vegetables, involved in the regulation of mitophagy, cell growth and cell death. Like other caloric restriction mimetics, spermidine is effective in cardioprotection, neuroprotection and anticancer immunosuppression by preserving mitochondrial function and control of autophagy.

Healthy reference population

Body mass excess

BFE

BME cutoffs

BMI

H

M

V_O₂max

mitObesity drugs

Publications: Q-junction

	Year	Reference	Organism	Tissue;cell	Stress	Diseases
Pallag 2022 MitoFit Proline	2022	Pallag G, Nazarian S, Ravasz D, Bui D, Komlódi T, Doerrier C, Gnaiger E, Seyfried TN, Chinopoulos C (2022) Proline oxidation leading to high electron flow through reduction of ubiquinone supports ATP production by F₁F_O-ATPase in mitochondria with inhibited Complex I. https://doi.org/10.26124/mitofit:2022-0001.v3 — 2022-05-04 published in https://doi.org/10.3390/ijms23095111	Mouse	Heart Kidney Liver Nervous system
Spielmann 2022 Mamm Genome	2022	Spielmann N, Schenkl C, Komlódi T, da Silva-Buttkus P, Heyne E, Rohde J, Amarie OV, Rathkolb B, Gnaiger E, Doenst T, Fuchs H, Gailus-Durner V, de Angelis MH, Szibor M (2022) Knockout of the Complex III subunit Uqcrh causes bioenergetic impairment and cardiac contractile dysfunction. Mamm Genome 10.1007/s00335-022-09973-w	Mouse	Heart	Oxidative stress;RONS Mitochondrial disease
Komlodi 2021 BEC Q	2021	Komlódi T, Cardoso LHD, Doerrier C, Moore AL, Rich PR, Gnaiger E (2021) Coupling and pathway control of coenzyme Q redox state and respiration in isolated mitochondria. https://doi.org/10.26124/bec:2021-0003	Mouse	Heart Nervous system
Spinazzi 2019 Proc Natl Acad Sci U S A	2019	Spinazzi M, Radaelli E, Horré K, Arranz AM, Gounko NV, Agostinis P, Maia TM, Impens F, Morais VA, Lopez-Lluch G, Serneels L, Navas P, De Strooper B (2019) PARL deficiency in mouse causes Complex III defects, coenzyme Q depletion, and Leigh-like syndrome. Proc Natl Acad Sci U S A 116:277-86.	Mouse	Nervous system		Neurodegenerative
Szibor 2019 Biochim Biophys Acta Bioenerg	2019	Szibor Marten, Gainutdinov Timur, Fernandez-Vizarra Erika, Dufour Eric, Gizatullina Zemfira, Debska-Vielhaber Grazyna, Heidler Juliana, Wittig Ilka, Viscomi Carlo, Gellerich Frank Norbert, Moore Anthony L (2019) Bioenergetic consequences from xenotopic expression of a tunicate AOX in mouse mitochondria: switch from RET and ROS to FET. Biochim Biophys Acta Bioenerg 1861:148137.	Mouse	Heart
Lemieux 2019 bioRxiv	2019	Lemieux H, Subarsky P, Doblander C, Wurm M, Troppmair J, Gnaiger E (2019) Mitochondrial respiratory function as an early biomarker of apoptosis induced by growth factor removal. bioRxiv doi: https://doi.org/10.1101/151480 .	Mouse	Blood cells	Cell death	Cancer
Cermakova 2019 Parasite	2019	Čermáková P, Kovalinka T, Ferenczyová K, Horváth A (2019) Coenzyme Q₂ is a universal substrate for the measurement of respiratory chain enzyme activities in trypanosomatids. Parasite 26:17.
Osakai 2019 Electrochemistry	2019	Osakai T, Yamamoto T, Ueki M (2019) Directional electron transfer from ubiquinone-10 to cytochrome c at a biomimetic self-assembled monolayer modified electrode. Electrochemistry 87:59-64.
Takahashi 2019 Arch Biochem Biophys	2019	Takahashi T, Mine Y, Okamoto T (2019) Extracellular coenzyme Q₁₀ (CoQ₁₀) is reduced to ubiquinol-10 by intact Hep G2 cells independent of intracellular CoQ₁₀ reduction. Arch Biochem Biophys 672:108067.
Martinez-Cifuentes 2017 Molecules	2017	Martínez-Cifuentes M, Salazar R, Ramírez-Rodríguez O, Weiss-López B, Araya-Maturana R (2017) Experimental and theoretical reduction potentials of some biologically active ortho-carbonyl para-quinones. Molecules 22:577.
Fragaki 2016 Biol Res	2016	Fragaki K, Chaussenot A, Benoist JF, Ait-El-Mkadem S, Bannwarth S, Rouzier C, Cochaud C, Paquis-Flucklinger V (2016) Coenzyme Q₁₀ defects may be associated with a deficiency of Q₁₀-independent mitochondrial respiratory chain complexes. Biol Res 49:4.
Gulaboski 2016 J Solid State Electrochem	2016	Gulaboski R, Markovski V, Jihe Z (2016) Redox chemistry of coenzyme Q—a short overview of the voltammetric features. J Solid State Electrochem 20:3229–3238.
Acosta 2016 Biochim Biophys Acta	2016	Acosta MJ, Vazquez Fonseca L, Desbats MA, Cerqua C, Zordan R, Trevisson E, Salviati L (2016) Coenzyme Q biosynthesis in health and disease. Biochim Biophys Acta 1857:1079-85.
García-Corzo 2015 Biochim Biophys Acta	2015	García-Corzo L, Luna-Sánchez M, Doerrier C, Ortiz F, Escames G, Acuña-Castroviejo D, López LC (2015) Ubiquinol-10 ameliorates mitochondrial encephalopathy associated with CoQ deficiency. Biochim Biophys Acta 1842:893-901.
Petrova 2014 Proc Chem	2014	Petrova EV, Korotkova EI, Kratochvil B, Voronova OA, Dorozhko EV, Bulycheva EV (2014) Investigation of coenzyme Q₁₀ by voltammetry. Proc Chem 10:173-8. https://doi.org/10.1016/j.proche.2014.10.030.
Enriquez 2014 Mol Syndromol	2014	Enriquez JA, Lenaz G (2014) Coenzyme Q and the respiratory chain: coenzyme Q pool and mitochondrial supercomplexes. Mol Syndromol 5:119-40.			Oxidative stress;RONS
La Guardia 2013 Front Physiol	2013	La Guardia PG, Alberici LC, Ravagnani FG, Catharino RR, Vercesi AE (2013) Protection of rat skeletal muscle fibers by either L-carnitine or coenzyme Q₁₀ against statins toxicity mediated by mitochondrial reactive oxygen generation. Front Physiol 4:103.	Rat	Skeletal muscle	Oxidative stress;RONS
Tang 2012 Methods Mol Biol	2012	Tang PH, Miles MV (2012) Measurement of oxidized and reduced coenzyme Q in biological fluids, cells, and tissues: an HPLC-EC method. Methods Mol Biol 837:149-68.
Song 2011 Free Radical Biol Med	2011	Song Y, Buettner GR (2011) Thermodynamic and kinetic considerations for the reaction of semiquinone radicals to form superoxide and hydrogen peroxide. Free Radical Biol Med 919-62.			Oxidative stress;RONS
Albury 2009 Physiol Plant	2009	Albury MS, Elliott C, Moore AL (2009) Towards a structural elucidation of the alternative oxidase in plants. Physiol Plant 137:316-27.
Ausili 2008 J Phys Chem B	2008	Ausili A, Torrecillas A, Aranda F, de Godos A, Sánchez-Bautista S, Corbalán-García S, Gómez-Fernández JC (2008) Redox state of coenzyme Q₁₀ determines its membrane localization. J Phys Chem B 112:12696-702.
Michalkiewicz 2007 Bioelectrochemistry	2007	Michalkiewicz S (2007) Cathodic reduction of coenzyme Q10 on glassy carbon electrode in acetic acid-acetonitrile solutions. Bioelectrochemistry 70:495-500.
Lenaz 2007 Am J Physiol Cell Physiol	2007	Lenaz G, Genova ML (2007) Kinetics of integrated electron transfer in the mitochondrial respiratory chain: random collisions vs. solid state electron channeling. Am J Physiol Cell Physiol 292:C1221-39. doi: 10.1152/ajpcell.00263.2006.
Pich 2002 Free Radic Res	2002	Pich MM, Castagnoli A, Biondi A, Bernacchia A, Tazzari PL, D'Aurelio M, Castelli GP, Formiggini G, Conte R, Bovina C, Lenaz G (2002) Ubiquinol and a coenzyme Q reducing system protect platelet mitochondrial function of transfusional buffy coats from oxidative stress. Free Radic Res 36:429-36.		Blood cells Platelet	Oxidative stress;RONS	Aging;senescence
Affourtit 2001 J Biol Chem	2001	Affourtit C, Krab K, Leach GR, Whitehouse DG, Moore AL (2001) New insights into the regulation of plant succinate dehydrogenase. On the role of the protonmotive force. J Biol Chem 276:32567-74.
Cooley 2001 J Bacteriol	2001	Cooley JW, Vermaas WFJ (2001) Succinate Dehydrogenase and Other Respiratory Pathways in Thylakoid Membranes of Synechocystis sp. Strain PCC 6803: Capacity Comparisons and Physiological Function. J Bacteriol 183:4251-8.
Goetz 2000 J Neural Transm (Vienna)	2000	Götz ME, Gerstner A, Harth R, Dirr A, Janetzky B, Kuhn W, Riederer P, Gerlach M (2000) Altered redox state of platelet coenzyme Q₁₀ in Parkinson's disease. J Neural Transm (Vienna) 107:41-8.
Affourtit 1999 J Biol Chem	1999	Affourtit C, Albury MS, Krab K, Moore AL (1999) Functional expression of the plant alternative oxidase affects growth of the yeast Schizosaccharomyces pombe. J Biol Chem 274:6212-8.
Wagner 1998 Plant Physiol	1998	Wagner AM, Wagner MJ, Moore AL (1998) In vivo ubiquinone reduction levels during thermogenesis in araceae. Plant Physiol 117:1501-6.
Meunier 1995 Biochemistry	1995	Meunier B, Madgwick SA, Reil E, Oettmeier W, Rich PR (1995) New inhibitors of the quinol oxidation sites of bacterial cytochromes bo and bd. Biochemistry 34:1076-83.
Rauchova 1995 Physiol Res	1995	Rauchová H, Drahota Z, Lenaz G (1995) Function of coenzyme Q in the cell: some biochemical and physiological properties. Physiol Res 44:209-16.
Van den Bergen 1994 Eur J Biochem	1994	Van den Bergen CW, Wagner AM, Krab K, Moore AL (1994) The relationship between electron flux and the redox poise of the quinone pool in plant mitochondria. Interplay between quinol-oxidizing and quinone-reducing pathways. Eur J Biochem 226:1071-8.
Moore 1991 Plant Physiol	1991	Moore AL, Dry IB, Wiskich JT (1991) Regulation of electron transport in plant mitochondria under state 4 conditions. Plant Physiol 95:34-40.
Day 1991 Plant Physiol	1991	Day DA, Dry IB, Soole KL, Wiskich JT, Moore AL (1991) Regulation of alternative pathway activity in plant mitochondria: deviations from Q-pool behavior during oxidation of NADH and quinols. Plant Physiol 95:948-53.
Zannoni 1990 FEBS Lett	1990	Zannoni D, Moore AL (1990) Measurement of the redox state of the ubiquinone pool in Rhodobacter capsulatus membrane fragments. FEBS Lett 271:123-7.
Dry 1989 Arch Biochem Biophys	1989	Dry IB, Moore AL, Day DA, Wiskich JT (1989) Regulation of alternative pathway activity in plant mitochondria: nonlinear relationship between electron flux and the redox poise of the quinone pool. Arch Biochem Biophys 273:148-57.
Moore 1988 FEBS Letters	1988	Moore AL, Dry IB, Wiskich TJ (1988) Measurement of the redox state of the ubiquinone pool in plant mitochondria. FEBS Lett 235:76-80.	Plants
Ragan 1985 Biochim Biophys Acta	1985	Ragan CI, Cottingham IR (1985) The kinetics of quinone pools in electron transport. Biochim Biophys Acta 811:13-31.
Rich 1984 Biochim Biophys Acta	1984	Rich PR (1984) Electron and proton transfers through quinones and cytochrome bc complexes. Biochim Biophys Acta 768:53-79.
Rich 1979 FEBS Lett	1979	Rich PR, Bendall DS (1979) A mechanism for the reduction of cytochromes by quinols in solution and its relevance to biological electron transfer reactions. FEBS Lett 105:189-94.
Mitchell 1979 Science	1979	Mitchell P (1979) Keilin’s respiratory chain concept and its chemiosmotic consequences. Science 206:1148-59.
Mitchell 1975 FEBS Letters	1975	Mitchell P (1975) The protonmotive Q cycle: A general formulation. FEBS Lett 59:137-9.
Kroeger 1973 Eur J Biochem	1973	Kröger A, Klingenberg M (1973) The kinetics of the redox reactions of ubiquinone related to the electron-transport activity in the respiratory chain. Eur J Biochem 34:358-68.	Bovines	Heart
Kroeger 1973b Eur J Biochem	1973	Kröger A, Klingenberg M (1973) Further evidence for the pool function of ubiquinone as derived from the inhibition of the electron transport by antimycin. Eur J Biochem 39:313-23.	Bovines	Heart
Gutman 1972 FEBS Lett	1972	Gutman M, Silman N (1972) Mutual inhibition between NADH oxidase and succinoxidase activities in respiring submitochondrial particles. FEBS Lett 26:207-10. doi: 10.1016/0014-5793(72)80574-x.
Gutman 1971 Biochemistry	1971	Gutman M, Coles CJ, Singer TP, Casida JE (1971) On the functional organization of the respiratory chain at the dehydrogenase-coenzyme Q junction. Biochemistry 10:2036-43.
Ernster 1969 Eur J Biochem	1969	Ernster L, Lee IY, Norling B, Persson B (1969) Studies with ubiquinone-depleted submitochondrial particles. Essentiality of ubiquinone for the interaction of succinate dehydrogenase, NADH dehydrogenase, and cytochrome b. Eur J Biochem 9:299-310.	Bovines	Heart
Mitchell 2011 Biochim Biophys Acta	1966	Mitchell P (1966) Chemiosmotic coupling in oxidative and photosynthetic phosphorylation. https://doi.org/10.1016/j.bbabio.2011.09.018
Kroeger 1966 Biochem Z	1966	Kröger A, Klingenberg M (1966) On the role of ubiquinone in mitochondria. II. Redox reactions of ubiquinone under the control of oxidative phosphorylation. Biochem Z 344:317-36.	Bovines	Heart

Abstracts: Q-junction

	Year	Reference	Organism	Tissue;cell	Stress	Diseases
Ravasz 2019 Abstract IOC141	2019	Ravasz D, Bui D, Kitayev A, Greenwood B, Hill C, Komlodi T, Doerrier C, Ozohanics O, Moore AL, Gnaiger E, Kiebish M, Kolev K, Seyfried TN, Willis WT, Narain N, Adam-Vizi V, Chinopoulos C (2019) Endogenous quinones sustain a moderate NADH oxidation by Complex I during anoxia. Mitochondr Physiol Network 24.02.	Mouse	Liver
Komlodi 2018b EBEC2018	2018	Endogenous quinones sustain NADH oxidation by Complex I during anoxia, supporting substrate-level phosphorylation in mouse liver mitochondria.	Mouse	Liver	Permeability transition Oxidative stress;RONS
Komlodi 2018 EBEC2018	2018	Electron supply to the Q-junction: assessment of mitochondrial respiration, H₂O₂ flux and the redox state of the Q-pool.	Mouse	Nervous system	Oxidative stress;RONS
Ravasz 2018 Abstract The evolving concept of mitochondria	2018	Vast pools of endogenous quinones sustain NADH oxidation by Complex I during anoxia, supporting substrate-level phosphorylation in mouse liver mitochondria.	Mouse	Liver		Other
Komlodi 2018 AussieMit	2018	Komlodi T, Hunger M, Moore AL, Gnaiger E (2018) Electron transfer at the Q-junction: new perspectives from combined measurement of mitochondrial O₂ flux, H₂O₂ flux, and coenzyme Q redox state. AussieMit 2018 Melbourne AU.	Mouse	Nervous system
Komlodi 2017 MiPschool Obergurgl	2017	Electron pressure exerted by convergent succinate- and glycerophosphate-pathways to the Q-junction regulate reversed electron transfer to Complex I and H₂O₂ production.	Mouse	Nervous system	Oxidative stress;RONS
Moore 2017 MiPschool Obergurgl	2017	The electron transfer-pathway – Q redox regulation and mitochondrial pathways to oxygen.

CoQ and mitObesity

Work in progress by Gnaiger E 2020-02-10 linked to a preprint in preparation on BME and mitObesity.