Human Metabolome Database Version 3.5

Showing metabocard for Ubiquinone-1 (HMDB02012)

Record Information
Version 3.5
Creation Date 2006-05-22 08:17:32 -0600
Update Date 2013-02-08 17:11:11 -0700
HMDB ID HMDB02012
Secondary Accession Numbers None
Metabolite Identification
Common Name Ubiquinone-1
Description Ubiquinone-1 is a member of the chemical class known as Polyprenylbenzoquinones. These are compounds containing a polyisoprene chain attached to a quinone at the second ring position. Ubiquione-1 has just 1 isoprene unit. Normally in humans it has 10. Ubiquinone-1 is an intermediate in the synthesis of Ubiquionone 10, which is also called Coenzyme Q (CoQ). CoQ is found in the membranes of endoplasmic reticulum, peroxisomes, lysosomes, vesicles and notably the inner membrane of the mitochondrion where it is an important part of the electron transport chain; there it passes reducing equivalents to acceptors such as Coenzyme Q : cytochrome c - oxidoreductase. CoQ is also essential in the formation of the apoptosome along with other adapter proteins. The loss of trophic factors activates pro-apoptotic enzymes, causing the breakdown of mitochondria. Because of its ability to transfer electrons and therefore act as an antioxidant, Coenzyme Q has become a valued dietary supplement. CoQ10 has been widely used for the treatment of heart disease (especially heart failure), gum diseases, and also breast cancer. The benzoquinone portion of Coenzyme Q10 is synthesized from amino acids, while the isoprene sidechain is synthesized from acetyl CoA through the mevalonate pathway. The mevalonate pathway is used for the first steps of cholesterol biosynthesis.
Structure Thumb
Download: MOL | SDF | PDB | SMILES | InChI
Display: 2D Structure | 3D Structure
Synonyms
  1. Coenzyme Q1
  2. CoQ1
  3. Ubiquinone Q1
  4. Ubiquinone-Q1
  5. Ubiquionone 1
Chemical Formula C14H18O4
Average Molecular Weight 250.2903
Monoisotopic Molecular Weight 250.120509064
IUPAC Name 2,3-dimethoxy-5-methyl-6-(3-methylbut-2-en-1-yl)cyclohexa-2,5-diene-1,4-dione
Traditional IUPAC Name ubiquinone-1
CAS Registry Number 727-81-1
SMILES COC1=C(OC)C(=O)C(CC=C(C)C)=C(C)C1=O
InChI Identifier InChI=1S/C14H18O4/c1-8(2)6-7-10-9(3)11(15)13(17-4)14(18-5)12(10)16/h6H,7H2,1-5H3
InChI Key SOECUQMRSRVZQQ-UHFFFAOYSA-N
Chemical Taxonomy
Kingdom Organic Compounds
Super Class Aliphatic Homomonocyclic Compounds
Class Benzoquinones
Sub Class N/A
Other Descriptors
  • Aliphatic Homomonocyclic Compounds
  • a ubiquinone(Cyc)
  • ubiquinones(ChEBI)
Substituents
  • Isoprene
  • Ketone
Direct Parent Benzoquinones
Ontology
Status Detected and Quantified
Origin
  • Endogenous
Biofunction
  • Anti-oxidant
  • Component of Ubiquinone biosynthesis
  • Electron donor
Application Not Available
Cellular locations
  • Membrane (predicted from logP)
Physical Properties
State Solid
Experimental Properties
Property Value Reference
Melting Point Not Available Not Available
Boiling Point Not Available Not Available
Water Solubility Not Available Not Available
LogP Not Available Not Available
Predicted Properties
Property Value Source
LogP 2.20 ALOGPS
LogP 2.22 ChemAxon
LogS -3.02 ALOGPS
pKa (strongest basic) -4.7 ChemAxon
Hydrogen Acceptor Count 4 ChemAxon
Hydrogen Donor Count 0 ChemAxon
Polar Surface Area 52.6 A2 ChemAxon
Rotatable Bond Count 4 ChemAxon
Refractivity 72.38 ChemAxon
Polarizability 26.94 ChemAxon
Formal Charge 0 ChemAxon
Physiological Charge 0 ChemAxon
Spectra
Not Available
Biological Properties
Cellular Locations
  • Membrane (predicted from logP)
Biofluid Locations
  • Blood
Tissue Location
  • Muscle
  • Skeletal Muscle
  • Fibroblasts
  • Intestine
  • Lymphocyte
  • Pancreas
  • Liver
  • Brain
  • Prostate
  • Nerve Cells
  • Platelet
Pathways
Name SMPDB Link KEGG Link
Ubiquinone Biosynthesis SMP00065 map00130 Link_out
Normal Concentrations
Biofluid Status Value Age Sex Condition Reference
Blood Detected and Quantified
0.00312 +/- 0.00032 uM Adult (>18 years old) Both Normal
Abnormal Concentrations
Biofluid Status Value Age Sex Condition Reference
Blood Detected and Quantified 0.00084 +/- 0.00040 uM Adult (>18 years old) Both Multiple sclerosis
Associated Disorders and Diseases
Disease References
Multiple sclerosis
  • Syburra C, Passi S: Oxidative stress in patients with multiple sclerosis. Ukr Biokhim Zh. 1999 May-Jun;71(3):112-5. Pubmed: 10609336 Link_out
      Associated OMIM IDs
      DrugBank ID Not Available
      DrugBank Metabolite ID Not Available
      Phenol Explorer Compound ID Not Available
      Phenol Explorer Metabolite ID Not Available
      FoodDB ID FDB022794
      KNApSAcK ID Not Available
      Chemspider ID 4307 Link_out
      KEGG Compound ID C00399 Link_out
      BioCyc ID Not Available
      BiGG ID Not Available
      Wikipedia Link Ubiquinone Link_out
      NuGOwiki Link HMDB02012 Link_out
      Metagene Link HMDB02012 Link_out
      METLIN ID 6435 Link_out
      PubChem Compound 4462 Link_out
      PDB ID UQ1 Link_out
      ChEBI ID 16389 Link_out
      References
      Synthesis Reference Naruta, Yoshinori; Maruyama, Kazuhiro. . Ubiquinone-1. Organic Syntheses (1993), 71 125-32.
      Material Safety Data Sheet (MSDS) Not Available
      General References
      1. Zhang Y, Eriksson M, Dallner G, Appelkvist EL: Analysis of ubiquinone and tocopherol levels in normal and hyperlipidemic human plasma. Lipids. 1998 Aug;33(8):811-5. Pubmed: 9727612 Link_out
      2. Karlsson J, Lin L, Gunnes S, Sylven C, Astrom H: Muscle ubiquinone in male effort angina patients. Mol Cell Biochem. 1996 Mar 23;156(2):173-8. Pubmed: 9095475 Link_out
      3. Passi S, Stancato A, Aleo E, Dmitrieva A, Littarru GP: Statins lower plasma and lymphocyte ubiquinol/ubiquinone without affecting other antioxidants and PUFA. Biofactors. 2003;18(1-4):113-24. Pubmed: 14695926 Link_out
      4. Karlsson J, Lin L, Sylven C, Jansson E: Muscle ubiquinone in healthy physically active males. Mol Cell Biochem. 1996 Mar 23;156(2):169-72. Pubmed: 9095474 Link_out
      5. 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 Jan;57(1):62-6. Pubmed: 7828383 Link_out
      6. Edlund C, Soderberg M, Kristensson K, Dallner G: Ubiquinone, dolichol, and cholesterol metabolism in aging and Alzheimer's disease. Biochem Cell Biol. 1992 Jun;70(6):422-8. Pubmed: 1449707 Link_out
      7. Pastore A, Giovamberardino GD, Bertini E, Tozzi G, Gaeta LM, Federici G, Piemonte F: Simultaneous determination of ubiquinol and ubiquinone in skeletal muscle of pediatric patients. Anal Biochem. 2005 Jul 15;342(2):352-5. Epub 2005 Mar 7. Pubmed: 15989930 Link_out
      8. Elmberger PG, Kalen A, Brunk UT, Dallner G: Discharge of newly-synthesized dolichol and ubiquinone with lipoproteins to rat liver perfusate and to the bile. Lipids. 1989 Nov;24(11):919-30. Pubmed: 2615561 Link_out
      9. Yamashita S, Yamamoto Y: Simultaneous detection of ubiquinol and ubiquinone in human plasma as a marker of oxidative stress. Anal Biochem. 1997 Jul 15;250(1):66-73. Pubmed: 9234900 Link_out
      10. Mancini A, Conte G, Milardi D, De Marinis L, Littarru GP: Relationship between sperm cell ubiquinone and seminal parameters in subjects with and without varicocele. Andrologia. 1998 Feb-Mar;30(1):1-4. Pubmed: 9567163 Link_out

      Enzymes

      Show all enzymes and transporters Important Only showing the first 50 enzymes. There are 56 enzymes in total.

      Name: NADH-ubiquinone oxidoreductase chain 1
      Reactions: Not Available
      Gene Name: MT-ND1
      Uniprot ID: P03886 Link_out
      Protein Sequence: FASTA
      Gene Sequence: FASTA
      Name: NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1
      Reactions: Not Available
      Gene Name: NDUFB1
      Uniprot ID: O75438 Link_out
      Protein Sequence: FASTA
      Gene Sequence: FASTA
      Name: NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial
      Reactions: Not Available
      Gene Name: NDUFS2
      Uniprot ID: O75306 Link_out
      Protein Sequence: FASTA
      Gene Sequence: FASTA
      Name: Succinate dehydrogenase cytochrome b560 subunit, mitochondrial
      Reactions: Not Available
      Gene Name: SDHC
      Uniprot ID: Q99643 Link_out
      Protein Sequence: FASTA
      Gene Sequence: FASTA
      Name: NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12
      Reactions: Not Available
      Gene Name: NDUFA12
      Uniprot ID: Q9UI09 Link_out
      Protein Sequence: FASTA
      Gene Sequence: FASTA
      Name: NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1
      Reactions: Not Available
      Gene Name: NDUFA1
      Uniprot ID: O15239 Link_out
      Protein Sequence: FASTA
      Gene Sequence: FASTA
      Name: NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 4-like 2
      Reactions: Not Available
      Gene Name: NDUFA4L2
      Uniprot ID: Q9NRX3 Link_out
      Protein Sequence: FASTA
      Gene Sequence: FASTA
      Name: NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3
      Reactions: Not Available
      Gene Name: NDUFA3
      Uniprot ID: O95167 Link_out
      Protein Sequence: FASTA
      Gene Sequence: FASTA
      Name: NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8
      Reactions: Not Available
      Gene Name: NDUFA8
      Uniprot ID: P51970 Link_out
      Protein Sequence: FASTA
      Gene Sequence: FASTA
      Name: NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 4
      Reactions: Not Available
      Gene Name: NDUFA4
      Uniprot ID: O00483 Link_out
      Protein Sequence: FASTA
      Gene Sequence: FASTA
      Name: NADH-ubiquinone oxidoreductase chain 4L
      Reactions: Not Available
      Gene Name: MT-ND4L
      Uniprot ID: P03901 Link_out
      Protein Sequence: FASTA
      Gene Sequence: FASTA
      Name: NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11
      Reactions: Not Available
      Gene Name: NDUFA11
      Uniprot ID: Q86Y39 Link_out
      Protein Sequence: FASTA
      Gene Sequence: FASTA
      Name: NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial
      Reactions: Not Available
      Gene Name: NDUFS4
      Uniprot ID: O43181 Link_out
      Protein Sequence: FASTA
      Gene Sequence: FASTA
      Name: NADH-ubiquinone oxidoreductase chain 5
      Reactions: Not Available
      Gene Name: MT-ND5
      Uniprot ID: P03915 Link_out
      Protein Sequence: FASTA
      Gene Sequence: FASTA
      Name: NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial
      Reactions: Not Available
      Gene Name: NDUFS3
      Uniprot ID: O75489 Link_out
      Protein Sequence: FASTA
      Gene Sequence: FASTA
      Name: NADH dehydrogenase [ubiquinone] iron-sulfur protein 5
      Reactions: Not Available
      Gene Name: NDUFS5
      Uniprot ID: O43920 Link_out
      Protein Sequence: FASTA
      Gene Sequence: FASTA
      Name: NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial
      Reactions: Not Available
      Gene Name: NDUFV2
      Uniprot ID: P19404 Link_out
      Protein Sequence: FASTA
      Gene Sequence: FASTA
      Name: NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10, mitochondrial
      Reactions: Not Available
      Gene Name: NDUFA10
      Uniprot ID: O95299 Link_out
      Protein Sequence: FASTA
      Gene Sequence: FASTA
      Name: NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6
      Reactions: Not Available
      Gene Name: NDUFA6
      Uniprot ID: P56556 Link_out
      Protein Sequence: FASTA
      Gene Sequence: FASTA
      Name: NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4
      Reactions: Not Available
      Gene Name: NDUFB4
      Uniprot ID: O95168 Link_out
      Protein Sequence: FASTA
      Gene Sequence: FASTA
      Name: Succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial
      Reactions: Not Available
      Gene Name: SDHA
      Uniprot ID: P31040 Link_out
      Protein Sequence: FASTA
      Gene Sequence: FASTA
      Name: NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial
      Reactions: Not Available
      Gene Name: NDUFS8
      Uniprot ID: O00217 Link_out
      Protein Sequence: FASTA
      Gene Sequence: FASTA
      Name: NADH-ubiquinone oxidoreductase chain 2
      Reactions: Not Available
      Gene Name: MT-ND2
      Uniprot ID: P03891 Link_out
      Protein Sequence: FASTA
      Gene Sequence: FASTA
      Name: NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 7
      Reactions: Not Available
      Gene Name: NDUFA7
      Uniprot ID: O95182 Link_out
      Protein Sequence: FASTA
      Gene Sequence: FASTA
      Name: NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2
      Reactions: Not Available
      Gene Name: NDUFA2
      Uniprot ID: O43678 Link_out
      Protein Sequence: FASTA
      Gene Sequence: FASTA
      Name: NADH-ubiquinone oxidoreductase 75 kDa subunit, mitochondrial
      Reactions: Not Available
      Gene Name: NDUFS1
      Uniprot ID: P28331 Link_out
      Protein Sequence: FASTA
      Gene Sequence: FASTA
      Name: NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 5, mitochondrial
      Reactions: Not Available
      Gene Name: NDUFB5
      Uniprot ID: O43674 Link_out
      Protein Sequence: FASTA
      Gene Sequence: FASTA
      Name: NADH dehydrogenase [ubiquinone] 1 subunit C2
      Reactions: Not Available
      Gene Name: NDUFC2
      Uniprot ID: O95298 Link_out
      Protein Sequence: FASTA
      Gene Sequence: FASTA
      Name: NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3
      Reactions: Not Available
      Gene Name: NDUFB3
      Uniprot ID: O43676 Link_out
      Protein Sequence: FASTA
      Gene Sequence: FASTA
      Name: NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7
      Reactions: Not Available
      Gene Name: NDUFB7
      Uniprot ID: P17568 Link_out
      Protein Sequence: FASTA
      Gene Sequence: FASTA
      Name: Acyl carrier protein, mitochondrial
      Reactions: Not Available
      Gene Name: NDUFAB1
      Uniprot ID: O14561 Link_out
      Protein Sequence: FASTA
      Gene Sequence: FASTA
      Name: NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5
      Reactions: Not Available
      Gene Name: NDUFA5
      Uniprot ID: Q16718 Link_out
      Protein Sequence: FASTA
      Gene Sequence: FASTA
      Name: Electron transfer flavoprotein-ubiquinone oxidoreductase, mitochondrial
      Reactions: Not Available
      Gene Name: ETFDH
      Uniprot ID: Q16134 Link_out
      Protein Sequence: FASTA
      Gene Sequence: FASTA
      Name: NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial
      Reactions: Not Available
      Gene Name: NDUFA9
      Uniprot ID: Q16795 Link_out
      Protein Sequence: FASTA
      Gene Sequence: FASTA
      Name: NADH-ubiquinone oxidoreductase chain 4
      Reactions: Not Available
      Gene Name: MT-ND4
      Uniprot ID: P03905 Link_out
      Protein Sequence: FASTA
      Gene Sequence: FASTA
      Name: Succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial
      Reactions: Not Available
      Gene Name: SDHD
      Uniprot ID: O14521 Link_out
      Protein Sequence: FASTA
      Gene Sequence: FASTA
      Name: NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial
      Reactions: Not Available
      Gene Name: NDUFS7
      Uniprot ID: O75251 Link_out
      Protein Sequence: FASTA
      Gene Sequence: FASTA
      Name: NADH-ubiquinone oxidoreductase chain 3
      Reactions: Not Available
      Gene Name: MT-ND3
      Uniprot ID: P03897 Link_out
      Protein Sequence: FASTA
      Gene Sequence: FASTA
      Name: NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial
      Reactions: Not Available
      Gene Name: NDUFV1
      Uniprot ID: P49821 Link_out
      Protein Sequence: FASTA
      Gene Sequence: FASTA
      Name: NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 9
      Reactions: Not Available
      Gene Name: NDUFB9
      Uniprot ID: Q9Y6M9 Link_out
      Protein Sequence: FASTA
      Gene Sequence: FASTA
      Name: NADH-ubiquinone oxidoreductase chain 6
      Reactions: Not Available
      Gene Name: MT-ND6
      Uniprot ID: P03923 Link_out
      Protein Sequence: FASTA
      Gene Sequence: FASTA
      Name: NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13
      Reactions: Not Available
      Gene Name: NDUFA13
      Uniprot ID: Q9P0J0 Link_out
      Protein Sequence: FASTA
      Gene Sequence: FASTA
      Name: NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial
      Reactions: Not Available
      Gene Name: NDUFS6
      Uniprot ID: O75380 Link_out
      Protein Sequence: FASTA
      Gene Sequence: FASTA
      Name: Succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial
      Reactions: Not Available
      Gene Name: SDHB
      Uniprot ID: P21912 Link_out
      Protein Sequence: FASTA
      Gene Sequence: FASTA
      Name: Hexaprenyldihydroxybenzoate methyltransferase, mitochondrial
      Reactions: Not Available
      Gene Name: COQ3
      Uniprot ID: Q9NZJ6 Link_out
      Protein Sequence: FASTA
      Gene Sequence: FASTA
      Name: Cytochrome b-c1 complex subunit 2, mitochondrial
      Reactions: Not Available
      Gene Name: UQCRC2
      Uniprot ID: P22695 Link_out
      Protein Sequence: FASTA
      Gene Sequence: FASTA
      Name: Cytochrome b-c1 complex subunit 9
      Reactions: Not Available
      Gene Name: UQCR10
      Uniprot ID: Q9UDW1 Link_out
      Protein Sequence: FASTA
      Gene Sequence: FASTA
      Name: Cytochrome b-c1 complex subunit 1, mitochondrial
      Reactions: Not Available
      Gene Name: UQCRC1
      Uniprot ID: P31930 Link_out
      Protein Sequence: FASTA
      Gene Sequence: FASTA
      Name: Cytochrome b-c1 complex subunit 7
      Reactions: Not Available
      Gene Name: UQCRB
      Uniprot ID: P14927 Link_out
      Protein Sequence: FASTA
      Gene Sequence: FASTA
      Name: Cytochrome b-c1 complex subunit Rieske, mitochondrial
      Reactions: Not Available
      Gene Name: UQCRFS1
      Uniprot ID: P47985 Link_out
      Protein Sequence: FASTA
      Gene Sequence: FASTA