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Human Metabolome Database Version 3.5

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Showing metabocard for QH2 (HMDB01304)

Record Information
Version 3.5
Creation Date 2005-11-16 08:48:42 -0700
Update Date 2013-01-28 19:29:45 -0700
HMDB ID HMDB01304
Secondary Accession Numbers None
Metabolite Identification
Common Name QH2
Description Reduced form of ubiquinone, a mobile electron transporter between complex I and III, or II and III of the electron transport chain. Both at complex I or complex II, two electrons and two protons are passed to ubiquinone reducing it to ubiquinol.
Structure Thumb
Download: MOL | SDF | SMILES | InChI
Display: 2D Structure | 3D Structure
Synonyms
  1. Coenzymes QH2
  2. CoQH2
  3. Reduced ubiquinone
  4. Ubiquinol
  5. Ubiquinone-1
Chemical Formula Not Available
Average Molecular Weight Not Available
Monoisotopic Molecular Weight Not Available
IUPAC Name Not Available
Traditional IUPAC Name Not Available
CAS Registry Number 56275-39-9
SMILES Not Available
InChI Identifier InChI=1S/C19H28O4/c1-12(2)8-7-9-13(3)10-11-15-14(4)16(20)18(22-5)19(23-6)17(15)21/h8,10,20-21H,7,9,11H2,1-6H3/b13-10+
InChI Key RNUCUWWMTTWKAH-JLHYYAGUSA-N
Chemical Taxonomy
Kingdom Organic Compounds
Super Class Lipids
Class Prenol Lipids
Sub Class Monoterpenes
Other Descriptors
  • Aromatic Homomonocyclic Compounds
  • Hydroxyquinols and Derivatives
  • Methoxyphenols and Derivatives
  • Monocyclic Monoterpenes
  • Pyrogallols and Derivatives
  • a ubiquinol(Cyc)
Substituents
  • Alkyl Aryl Ether
  • Anisole
  • Hydroquinone
  • Isoprene
  • M Cresol
  • O Cresol
  • Phenol
  • Phenol Derivative
  • Toluene
Direct Parent Aromatic Monoterpenes
Ontology
Status Detected and Quantified
Origin
  • Endogenous
  • Food
Biofunction
  • Cell signaling
  • Component of Ubiquinone biosynthesis
  • Fuel and energy storage
  • Fuel or energy source
  • Membrane integrity/stability
Application
  • Nutrients
  • Stabilizers
  • Surfactants and Emulsifiers
Cellular locations
  • Extracellular
  • Membrane
  • Inner mitochondrial membrane
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
Formal Charge 0 ChemAxon
Spectra
Not Available
Biological Properties
Cellular Locations
  • Extracellular
  • Membrane
  • Inner mitochondrial membrane
Biofluid Locations
  • Blood
Tissue Location
  • Muscle
  • Skeletal Muscle
  • Testes
  • Epidermis
Pathways Not Available
Normal Concentrations
Not Available
Abnormal Concentrations
Biofluid Status Value Age Sex Condition Comments
Blood Detected and Quantified
Article_icon
7.4 +/- 2.7 uM Adult (>18 years old) Both Heart failure Congestive heart failure
Associated Disorders and Diseases
Disease References
Heart failure
  • Bhagavan HN, Chopra RK: Plasma coenzyme Q10 response to oral ingestion of coenzyme Q10 formulations. Mitochondrion. 2007 Jun;7 Suppl:S78-88. Epub 2007 Mar 27. Pubmed: 17482886 Link_out
    Associated OMIM IDs None
    DrugBank ID Not Available
    Phenol Explorer Compound ID Not Available
    Phenol Explorer Metabolite ID Not Available
    FoodDB ID FDB022543
    KNApSAcK ID Not Available
    Chemspider ID 4444052 Link_out
    KEGG Compound ID C00390 Link_out
    BioCyc ID QH2 Link_out
    BiGG ID Not Available
    Wikipedia Link ubiquinol Link_out
    NuGOwiki Link HMDB01304 Link_out
    Metagene Link HMDB01304 Link_out
    METLIN ID 6146 Link_out
    PubChem Compound 5280344 Link_out
    PDB ID Not Available
    ChEBI ID Not Available
    References
    Synthesis Reference Not Available
    Material Safety Data Sheet (MSDS) Not Available
    General References
    1. Shindo Y, Witt E, Han D, Epstein W, Packer L: Enzymic and non-enzymic antioxidants in epidermis and dermis of human skin. J Invest Dermatol. 1994 Jan;102(1):122-4. Pubmed: 8288904 Link_out
    2. Passi S, Grandinetti M, Maggio F, Stancato A, De Luca C: Epidermal oxidative stress in vitiligo. Pigment Cell Res. 1998 Apr;11(2):81-5. Pubmed: 9585244 Link_out
    3. Neuzil J, Stocker R: Free and albumin-bound bilirubin are efficient co-antioxidants for alpha-tocopherol, inhibiting plasma and low density lipoprotein lipid peroxidation. J Biol Chem. 1994 Jun 17;269(24):16712-9. Pubmed: 8206992 Link_out
    4. Mohr D, Umeda Y, Redgrave TG, Stocker R: Antioxidant defenses in rat intestine and mesenteric lymph. Redox Rep. 1999;4(3):79-87. Pubmed: 10496410 Link_out
    5. Kontush A, Spranger T, Reich A, Baum K, Beisiegel U: Lipophilic antioxidants in blood plasma as markers of atherosclerosis: the role of alpha-carotene and gamma-tocopherol. Atherosclerosis. 1999 May;144(1):117-22. Pubmed: 10381285 Link_out
    6. Halliwell B, Hu ML, Louie S, Duvall TR, Tarkington BK, Motchnik P, Cross CE: Interaction of nitrogen dioxide with human plasma. Antioxidant depletion and oxidative damage. FEBS Lett. 1992 Nov 16;313(1):62-6. Pubmed: 1426270 Link_out
    7. Peltola V, Huhtaniemi I, Metsa-Ketela T, Ahotupa M: Induction of lipid peroxidation during steroidogenesis in the rat testis. Endocrinology. 1996 Jan;137(1):105-12. Pubmed: 8536600 Link_out
    8. Miles L, Miles MV, Tang PH, Horn PS, Wong BL, DeGrauw TJ, Morehart PJ, Bove KE: Muscle coenzyme Q: a potential test for mitochondrial activity and redox status. Pediatr Neurol. 2005 May;32(5):318-24. Pubmed: 15866432 Link_out
    9. Lonnrot K, Metsa-Ketela T, Molnar G, Ahonen JP, Latvala M, Peltola J, Pietila T, Alho H: The effect of ascorbate and ubiquinone supplementation on plasma and CSF total antioxidant capacity. Free Radic Biol Med. 1996;21(2):211-7. Pubmed: 8818636 Link_out
    10. Kontush A, Reich A, Baum K, Spranger T, Finckh B, Kohlschutter A, Beisiegel U: Plasma ubiquinol-10 is decreased in patients with hyperlipidaemia. Atherosclerosis. 1997 Feb 28;129(1):119-26. Pubmed: 9069526 Link_out
    11. Hargreaves P, Rahman S, Guthrie P, Taanman JW, Leonard JV, Land JM, Heales SJ: Diagnostic value of succinate ubiquinone reductase activity in the identification of patients with mitochondrial DNA depletion. J Inherit Metab Dis. 2002 Feb;25(1):7-16. Pubmed: 12004863 Link_out
    Enzymes
    Name: NADH-ubiquinone oxidoreductase chain 1
    Reactions:
    • NADH + H+ + ubiquinone = NAD+ + ubiquinol [RN:R02163]
    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:
    • --- []
    Gene Name: NDUFB1
    Uniprot ID: O75438 Link_out
    Protein Sequence: FASTA
    Gene Sequence: FASTA
    Name: NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial
    Reactions:
    • NADH + H+ + acceptor = NAD+ + reduced acceptor [RN:R00281]
    Gene Name: NDUFS2
    Uniprot ID: O75306 Link_out
    Protein Sequence: FASTA
    Gene Sequence: FASTA
    Name: Succinate dehydrogenase cytochrome b560 subunit, mitochondrial
    Reactions:
    • --- []
    Gene Name: SDHC
    Uniprot ID: Q99643 Link_out
    Protein Sequence: FASTA
    Gene Sequence: FASTA
    Name: NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12
    Reactions:
    • --- []
    Gene Name: NDUFA12
    Uniprot ID: Q9UI09 Link_out
    Protein Sequence: FASTA
    Gene Sequence: FASTA
    Name: NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1
    Reactions:
    • --- []
    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:
      Gene Name: NDUFA4L2
      Uniprot ID: Q9NRX3 Link_out
      Protein Sequence: FASTA
      Gene Sequence: FASTA
      Name: NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3
      Reactions:
      • --- []
      Gene Name: NDUFA3
      Uniprot ID: O95167 Link_out
      Protein Sequence: FASTA
      Gene Sequence: FASTA
      Name: NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8
      Reactions:
      • --- []
      Gene Name: NDUFA8
      Uniprot ID: P51970 Link_out
      Protein Sequence: FASTA
      Gene Sequence: FASTA
      Name: NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 4
      Reactions:
      • --- []
      Gene Name: NDUFA4
      Uniprot ID: O00483 Link_out
      Protein Sequence: FASTA
      Gene Sequence: FASTA
      Name: NADH-ubiquinone oxidoreductase chain 4L
      Reactions:
      • NADH + H+ + ubiquinone = NAD+ + ubiquinol [RN:R02163]
      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:
      • --- []
      Gene Name: NDUFA11
      Uniprot ID: Q86Y39 Link_out
      Protein Sequence: FASTA
      Gene Sequence: FASTA
      Name: NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial
      Reactions:
      • --- []
      Gene Name: NDUFS4
      Uniprot ID: O43181 Link_out
      Protein Sequence: FASTA
      Gene Sequence: FASTA
      Name: NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial
      Reactions:
      • NADH + H+ + acceptor = NAD+ + reduced acceptor [RN:R00281]
      Gene Name: NDUFS3
      Uniprot ID: O75489 Link_out
      Protein Sequence: FASTA
      Gene Sequence: FASTA
      Name: NADH dehydrogenase [ubiquinone] iron-sulfur protein 5
      Reactions:
      • --- []
      Gene Name: NDUFS5
      Uniprot ID: O43920 Link_out
      Protein Sequence: FASTA
      Gene Sequence: FASTA
      Name: NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial
      Reactions:
      • NADH + H+ + acceptor = NAD+ + reduced acceptor [RN:R00281]
      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:
      • --- []
      Gene Name: NDUFA10
      Uniprot ID: O95299 Link_out
      Protein Sequence: FASTA
      Gene Sequence: FASTA
      Name: NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6
      Reactions:
      • --- []
      Gene Name: NDUFA6
      Uniprot ID: P56556 Link_out
      Protein Sequence: FASTA
      Gene Sequence: FASTA
      Name: NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4
      Reactions:
      • --- []
      Gene Name: NDUFB4
      Uniprot ID: O95168 Link_out
      Protein Sequence: FASTA
      Gene Sequence: FASTA
      Name: Succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial
      Reactions:
      • succinate + ubiquinone = fumarate + ubiquinol [RN:R02164]
      Gene Name: SDHA
      Uniprot ID: P31040 Link_out
      Protein Sequence: FASTA
      Gene Sequence: FASTA
      Name: NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial
      Reactions:
      • NADH + H+ + acceptor = NAD+ + reduced acceptor [RN:R00281]
      Gene Name: NDUFS8
      Uniprot ID: O00217 Link_out
      Protein Sequence: FASTA
      Gene Sequence: FASTA
      Name: NADH-ubiquinone oxidoreductase chain 2
      Reactions:
      • NADH + H+ + ubiquinone = NAD+ + ubiquinol [RN:R02163]
      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:
      • --- []
      Gene Name: NDUFA7
      Uniprot ID: O95182 Link_out
      Protein Sequence: FASTA
      Gene Sequence: FASTA
      Name: NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2
      Reactions:
      • --- []
      Gene Name: NDUFA2
      Uniprot ID: O43678 Link_out
      Protein Sequence: FASTA
      Gene Sequence: FASTA
      Name: NADH-ubiquinone oxidoreductase 75 kDa subunit, mitochondrial
      Reactions:
      • NADH + H+ + acceptor = NAD+ + reduced acceptor [RN:R00281]
      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:
      • --- []
      Gene Name: NDUFB5
      Uniprot ID: O43674 Link_out
      Protein Sequence: FASTA
      Gene Sequence: FASTA
      Name: NADH dehydrogenase [ubiquinone] 1 subunit C2
      Reactions:
      • --- []
      Gene Name: NDUFC2
      Uniprot ID: O95298 Link_out
      Protein Sequence: FASTA
      Gene Sequence: FASTA
      Name: NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3
      Reactions:
      • --- []
      Gene Name: NDUFB3
      Uniprot ID: O43676 Link_out
      Protein Sequence: FASTA
      Gene Sequence: FASTA
      Name: NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7
      Reactions:
      • --- []
      Gene Name: NDUFB7
      Uniprot ID: P17568 Link_out
      Protein Sequence: FASTA
      Gene Sequence: FASTA
      Name: Acyl carrier protein, mitochondrial
      Reactions:
      • --- []
      Gene Name: NDUFAB1
      Uniprot ID: O14561 Link_out
      Protein Sequence: FASTA
      Gene Sequence: FASTA
      Name: NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5
      Reactions:
      • --- []
      Gene Name: NDUFA5
      Uniprot ID: Q16718 Link_out
      Protein Sequence: FASTA
      Gene Sequence: FASTA
      Name: Electron transfer flavoprotein-ubiquinone oxidoreductase, mitochondrial
      Reactions:
      • reduced electron-transferring flavoprotein + ubiquinone = electron-transferring flavoprotein + ubiquinol [RN:R04433]
      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:
      • --- []
      Gene Name: NDUFA9
      Uniprot ID: Q16795 Link_out
      Protein Sequence: FASTA
      Gene Sequence: FASTA
      Name: NADH-ubiquinone oxidoreductase chain 4
      Reactions:
      • NADH + H+ + ubiquinone = NAD+ + ubiquinol [RN:R02163]
      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:
        Gene Name: SDHD
        Uniprot ID: O14521 Link_out
        Protein Sequence: FASTA
        Gene Sequence: FASTA
        Name: NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial
        Reactions:
        • NADH + H+ + acceptor = NAD+ + reduced acceptor [RN:R00281]
        Gene Name: NDUFS7
        Uniprot ID: O75251 Link_out
        Protein Sequence: FASTA
        Gene Sequence: FASTA
        Name: NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial
        Reactions:
        • NADH + H+ + acceptor = NAD+ + reduced acceptor [RN:R00281]
        Gene Name: NDUFV1
        Uniprot ID: P49821 Link_out
        Protein Sequence: FASTA
        Gene Sequence: FASTA
        Name: NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 9
        Reactions:
        • --- []
        Gene Name: NDUFB9
        Uniprot ID: Q9Y6M9 Link_out
        Protein Sequence: FASTA
        Gene Sequence: FASTA
        Name: NADH-ubiquinone oxidoreductase chain 6
        Reactions:
        • NADH + H+ + ubiquinone = NAD+ + ubiquinol [RN:R02163]
        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:
        • --- []
        Gene Name: NDUFA13
        Uniprot ID: Q9P0J0 Link_out
        Protein Sequence: FASTA
        Gene Sequence: FASTA
        Name: NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial
        Reactions:
        • --- []
        Gene Name: NDUFS6
        Uniprot ID: O75380 Link_out
        Protein Sequence: FASTA
        Gene Sequence: FASTA
        Name: Succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial
        Reactions:
        • succinate + ubiquinone = fumarate + ubiquinol [RN:R02164]
        Gene Name: SDHB
        Uniprot ID: P21912 Link_out
        Protein Sequence: FASTA
        Gene Sequence: FASTA
        Name: Cytochrome b-c1 complex subunit 2, mitochondrial
        Reactions:
          Gene Name: UQCRC2
          Uniprot ID: P22695 Link_out
          Protein Sequence: FASTA
          Gene Sequence: FASTA
          Name: Cytochrome b-c1 complex subunit 9
          Reactions:
          • --- []
          Gene Name: UQCR10
          Uniprot ID: Q9UDW1 Link_out
          Protein Sequence: FASTA
          Gene Sequence: FASTA
          Name: Cytochrome b-c1 complex subunit 1, mitochondrial
          Reactions:
            Gene Name: UQCRC1
            Uniprot ID: P31930 Link_out
            Protein Sequence: FASTA
            Gene Sequence: FASTA
            Name: Cytochrome b-c1 complex subunit 7
            Reactions:
              Gene Name: UQCRB
              Uniprot ID: P14927 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Cytochrome b-c1 complex subunit Rieske, mitochondrial
              Reactions:
              • QH2 + 2 ferricytochrome c = Q + 2 ferrocytochrome c + 2 H+ [RN:R02161]
              Gene Name: UQCRFS1
              Uniprot ID: P47985 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Cytochrome c1, heme protein, mitochondrial
              Reactions:
                Gene Name: CYC1
                Uniprot ID: P08574 Link_out
                Protein Sequence: FASTA
                Gene Sequence: FASTA
                Name: Cytochrome b-c1 complex subunit 6, mitochondrial
                Reactions:
                  Gene Name: UQCRH
                  Uniprot ID: P07919 Link_out
                  Protein Sequence: FASTA
                  Gene Sequence: FASTA
                  Name: NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial
                  Reactions:
                    Gene Name: NDUFB11
                    Uniprot ID: Q9NX14 Link_out
                    Protein Sequence: FASTA
                    Gene Sequence: FASTA
                    Name: Cytochrome b-c1 complex subunit 8
                    Reactions:
                      Gene Name: UQCRQ
                      Uniprot ID: O14949 Link_out
                      Protein Sequence: FASTA
                      Gene Sequence: FASTA
                      Name: Cytochrome b-c1 complex subunit 10
                      Reactions:
                        Gene Name: UQCR11
                        Uniprot ID: O14957 Link_out
                        Protein Sequence: FASTA
                        Gene Sequence: FASTA
                        Name: NADH dehydrogenase (Ubiquinone) 1 beta subcomplex, 2, 8kDa
                        Reactions:
                          Gene Name: NDUFB2
                          Uniprot ID: A4D1T5 Link_out
                          Protein Sequence: FASTA
                          Gene Sequence: FASTA