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Human Metabolome Database Version 3.5

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Showing metabocard for Fumaric acid (HMDB00134)

Record Information
Version 3.5
Creation Date 2005-11-16 08:48:42 -0700
Update Date 2013-02-08 17:07:57 -0700
HMDB ID HMDB00134
Secondary Accession Numbers None
Metabolite Identification
Common Name Fumaric acid
Description Fumaric acid is a precursor to L-malate in the Krebs tricarboxylic acid cycle. It is formed by the oxidation of succinate by succinate dehydrogenase. Fumarate is converted by fumarase to malate. A fumarate is a salt or ester of the organic compound fumaric acid, a dicarboxylic acid. (wikipedia).
Structure Thumb
Download: MOL | SDF | SMILES | InChI
Display: 2D Structure | 3D Structure
Synonyms
  1. (2E)-But-2-enedioate
  2. (2E)-But-2-enedioic acid
  3. (E)-2-Butenedioate
  4. (E)-2-Butenedioic acid
  5. 2-(E)-Butenedioate
  6. 2-(E)-Butenedioic acid
  7. Allomaleate
  8. Allomaleic acid
  9. Boletate
  10. Boletic acid
  11. FC 33
  12. Fumarate
  13. Fumaric acid
  14. Lichenate
  15. Lichenic acid
  16. Sodium fumarate
  17. trans-1,2-Ethylenedicarboxylate
  18. trans-1,2-Ethylenedicarboxylic acid
  19. trans-2-Butenedioate
  20. trans-2-Butenedioic acid
  21. trans-Butenedioate
  22. trans-Butenedioic acid
Chemical Formula C4H4O4
Average Molecular Weight 116.0722
Monoisotopic Molecular Weight 116.010958616
IUPAC Name (2E)-but-2-enedioic acid
Traditional IUPAC Name maleic acid
CAS Registry Number 110-17-8
SMILES OC(=O)\C=C\C(O)=O
InChI Identifier InChI=1S/C4H4O4/c5-3(6)1-2-4(7)8/h1-2H,(H,5,6)(H,7,8)/b2-1+
InChI Key VZCYOOQTPOCHFL-OWOJBTEDSA-N
Chemical Taxonomy
Kingdom Organic Compounds
Super Class Lipids
Class Fatty Acids and Conjugates
Sub Class Unsaturated Fatty Acids
Other Descriptors
  • Aliphatic Acyclic Compounds
  • Dicarboxylic Acids and Derivatives
  • Organic Compounds
  • butenedioic acid(ChEBI)
Substituents
  • Acyclic Alkene
  • Allyl Alcohol
  • Carboxylic Acid
  • Enone
Direct Parent Unsaturated Fatty Acids
Ontology
Status Detected and Quantified
Origin
  • Endogenous
  • Food
Biofunction
  • Cell signaling
  • Fuel and energy storage
  • Fuel or energy source
  • Membrane integrity/stability
Application
  • Nutrients
  • Stabilizers
  • Surfactants and Emulsifiers
Cellular locations
  • Extracellular
  • Membrane
  • Mitochondria
Physical Properties
State Solid
Experimental Properties
Property Value Reference
Melting Point 549 °C Not Available
Boiling Point Not Available Not Available
Water Solubility 7.0 mg/mL Not Available
LogP 0.46 HANSCH,C ET AL. (1995)
Predicted Properties
Property Value Source
Water Solubility 24.1 g/L ALOGPS
LogP 0.21 ALOGPS
LogP -0.041 ChemAxon
LogS -0.68 ALOGPS
pKa (strongest acidic) 3.55 ChemAxon
Hydrogen Acceptor Count 4 ChemAxon
Hydrogen Donor Count 2 ChemAxon
Polar Surface Area 74.6 A2 ChemAxon
Rotatable Bond Count 2 ChemAxon
Refractivity 24.61 ChemAxon
Polarizability 9.35 ChemAxon
Formal Charge 0 ChemAxon
Physiological Charge -2 ChemAxon
Spectra
Gas-MS Spectrum
1H NMR Spectrum
13C NMR Spectrum
MS/MS Spectrum Quattro_QQQ 10
MS/MS Spectrum Quattro_QQQ 25
MS/MS Spectrum Quattro_QQQ 40
MS/MS Spectrum EI-B (HITACHI RMU-6L)
MS/MS Spectrum GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies)
MS/MS Spectrum GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies)
MS/MS Spectrum GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies)
MS/MS Spectrum GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies )
MS/MS Spectrum GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies)
MS/MS Spectrum GC-MS
[1H,1H] 2D NMR Spectrum
[1H,13C] 2D NMR Spectrum
Biological Properties
Cellular Locations
  • Extracellular
  • Membrane
  • Mitochondria
Biofluid Locations
  • Blood
  • Cerebrospinal Fluid (CSF)
  • Urine
Tissue Location
  • Prostate
Pathways
Name SMPDB Link KEGG Link
Phenylalanine and Tyrosine Metabolism SMP00008 map00360 Link_out
Arginine and Proline Metabolism SMP00020 map00330 Link_out
Aspartate Metabolism SMP00067 map00250 Link_out
Urea Cycle SMP00059 map00330 Link_out
Mitochondrial Electron Transport Chain SMP00355 map00190 Link_out
Tyrosine Metabolism SMP00006 map00350 Link_out
Citric Acid Cycle SMP00057 map00020 Link_out
Normal Concentrations
Biofluid Status Value Age Sex Condition Comments
Blood Detected and Quantified
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1.5 (0.0-4.0) uM Adult (>18 years old) Both Normal Not Available
Cerebrospinal Fluid (CSF) Detected and Quantified
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1 uM Children (1-13 year old) Both Normal Not Available
Cerebrospinal Fluid (CSF) Detected and Quantified
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33.0 (9.00-57.0) uM Adult (>18 years old) Both Normal Not Available
Urine Detected and Quantified
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1.0 (0.0-2.0) umol/mmol creatinine Adult (>18 years old) Both Normal Not Available
Urine Detected and Quantified
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0.7 (0.2-1.7) umol/mmol creatinine Adult (>18 years old) Both Normal urine by NMR
Urine Detected and Quantified
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0.4 (0.2-0.8) umol/mmol creatinine Adult (>18 years old) Both Normal Not Available
Urine Detected and Quantified
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0.1-0.4 umol/mmol creatinine Adult (>18 years old) Both Normal Not Available
Urine Detected and Quantified
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0.1 (0.1-1.7) umol/mmol creatinine Children (1-13 year old) Both Normal Not Available
Urine Detected and Quantified
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0.2 umol/mmol creatinine Adult (>18 years old) Both Normal Not Available
Urine Detected and Quantified
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0.95 +/- 0.93 umol/mmol creatinine Adult (>18 years old) Both Normal Not Available
Urine Detected and Quantified
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10.4 (2.8-53.7) umol/mmol creatinine Newborn (0-30 days old) Both Normal Not Available
Urine Detected and Quantified
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0.5 (0.1-1.7) umol/mmol creatinine Adolescent (13-18 years old) Both Normal Not Available
Urine Detected and Quantified
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10.7 (0.1-28.2) umol/mmol creatinine Infant (0-1 year old) Both Normal Not Available
Urine Detected and not Quantified
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Not Applicable Adult (>18 years old) Male Normal Not Available
Abnormal Concentrations
Biofluid Status Value Age Sex Condition Comments
Blood Detected and Quantified
Article_icon
2.0 (0.0-4.0) uM Adult (>18 years old) Both Fumaric aciduria Not Available
Blood Detected and Quantified
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6.0 (4.0-8.0) uM Adult (>18 years old) Both Fumaric Aciduria Not Available
Cerebrospinal Fluid (CSF) Detected and Quantified
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11.0 (2.00-20.0) uM Elderly (>65 years old) Both Alzheimer's disease Not Available
Urine Detected and Quantified
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3500.0 (3000.0-4000.0) umol/mmol creatinine Adult (>18 years old) Both Fumaric Aciduria Not Available
Urine Detected and Quantified
0.7 (0.0-4.0) umol/mmol creatinine Adult (>18 years old) Both Lung cancer Not Available
Associated Disorders and Diseases
Disease References
Alzheimer's disease
  • Redjems-Bennani N, Jeandel C, Lefebvre E, Blain H, Vidailhet M, Gueant JL: Abnormal substrate levels that depend upon mitochondrial function in cerebrospinal fluid from Alzheimer patients. Gerontology. 1998;44(5):300-4. Pubmed: 9693263 Link_out
    Lung Cancer
        Fumarase deficiency
          • http://www.metagene.de/program/d.prg?mp=FUMARIC%20ACIDURIA
          Associated OMIM IDs
          DrugBank ID DB01677 Link_out
          Phenol Explorer Compound ID Not Available
          Phenol Explorer Metabolite ID Not Available
          FoodDB ID FDB003291
          KNApSAcK ID C00001183 Link_out
          Chemspider ID 10197150 Link_out
          KEGG Compound ID C00122 Link_out
          BioCyc ID FUM Link_out
          BiGG ID 33938 Link_out
          Wikipedia Link Fumaric acid Link_out
          NuGOwiki Link HMDB00134 Link_out
          Metagene Link HMDB00134 Link_out
          METLIN ID 3242 Link_out
          PubChem Compound 444972 Link_out
          PDB ID FUM Link_out
          ChEBI ID 18012 Link_out
          References
          Synthesis Reference Dong, Changsheng; Ma, Xinming. Method for preparation of fumaric acid from the tail gas acid spray solution from oxidation of phthalic anhydride. Faming Zhuanli Shenqing Gongkai Shuomingshu (2007), 5pp.
          Material Safety Data Sheet (MSDS) Download (PDF)
          General References
          1. Guneral F, Bachmann C: Age-related reference values for urinary organic acids in a healthy Turkish pediatric population. Clin Chem. 1994 Jun;40(6):862-6. Pubmed: 8087979 Link_out
          2. Redjems-Bennani N, Jeandel C, Lefebvre E, Blain H, Vidailhet M, Gueant JL: Abnormal substrate levels that depend upon mitochondrial function in cerebrospinal fluid from Alzheimer patients. Gerontology. 1998;44(5):300-4. Pubmed: 9693263 Link_out
          3. Shoemaker JD, Elliott WH: Automated screening of urine samples for carbohydrates, organic and amino acids after treatment with urease. J Chromatogr. 1991 Jan 2;562(1-2):125-38. Pubmed: 2026685 Link_out
          4. Hoffmann GF, Meier-Augenstein W, Stockler S, Surtees R, Rating D, Nyhan WL: Physiology and pathophysiology of organic acids in cerebrospinal fluid. J Inherit Metab Dis. 1993;16(4):648-69. Pubmed: 8412012 Link_out
          5. Sreekumar A, Poisson LM, Rajendiran TM, Khan AP, Cao Q, Yu J, Laxman B, Mehra R, Lonigro RJ, Li Y, Nyati MK, Ahsan A, Kalyana-Sundaram S, Han B, Cao X, Byun J, Omenn GS, Ghosh D, Pennathur S, Alexander DC, Berger A, Shuster JR, Wei JT, Varambally S, Beecher C, Chinnaiyan AM: Metabolomic profiles delineate potential role for sarcosine in prostate cancer progression. Nature. 2009 Feb 12;457(7231):910-4. Pubmed: 19212411 Link_out
          Enzymes
          Name: Succinate dehydrogenase cytochrome b560 subunit, mitochondrial
          Reactions:
          • --- []
          Gene Name: SDHC
          Uniprot ID: Q99643 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: Succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial
          Reactions:
            Gene Name: SDHD
            Uniprot ID: O14521 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: Dopamine beta-hydroxylase
            Reactions:
            • 3,4-dihydroxyphenethylamine + ascorbate + O2 = noradrenaline + dehydroascorbate + H2O [RN:R02535]
            Gene Name: DBH
            Uniprot ID: P09172 Link_out
            Protein Sequence: FASTA
            Gene Sequence: FASTA
            Name: Adenylosuccinate lyase
            Reactions:
            • (1) N6-(1,2-dicarboxyethyl)AMP = fumarate + AMP [RN:R01083]
            • (2) (S)-2-[5-amino-1-(5-phospho-D-ribosyl)imidazole-4- carboxamido]succinate = fumarate + 5-amino-1-(5-phospho-D-ribosyl)imidazole-4-carboxamide [RN:R04559]
            Gene Name: ADSL
            Uniprot ID: P30566 Link_out
            Protein Sequence: FASTA
            Gene Sequence: FASTA
            Name: Argininosuccinate lyase
            Reactions:
            • 2-(Nomega-L-arginino)succinate = fumarate + L-arginine [RN:R01086]
            Gene Name: ASL
            Uniprot ID: P04424 Link_out
            Protein Sequence: FASTA
            Gene Sequence: FASTA
            Name: Fumarylacetoacetase
            Reactions:
            • 4-fumarylacetoacetate + H2O = acetoacetate + fumarate [RN:R01364]
            Gene Name: FAH
            Uniprot ID: P16930 Link_out
            Protein Sequence: FASTA
            Gene Sequence: FASTA
            Name: Fumarate hydratase, mitochondrial
            Reactions:
            • (S)-malate = fumarate + H2O [RN:R01082]
            Gene Name: FH
            Uniprot ID: P07954 Link_out
            Protein Sequence: FASTA
            Gene Sequence: FASTA
            Name: NAD-dependent malic enzyme, mitochondrial
            Reactions:
            • (S)-malate + NAD+ = pyruvate + CO2 + NADH [RN:R00214]
            Gene Name: ME2
            Uniprot ID: P23368 Link_out
            Protein Sequence: FASTA
            Gene Sequence: FASTA
            Name: Fumarate hydratase
            Reactions:
              Gene Name: FH
              Uniprot ID: B1ANK7 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA