Human Metabolome Database Version 3.5

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 | PDB | 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 Reference
Blood Detected and Quantified
1.5 (0.0-4.0) uM Adult (>18 years old) Both Normal
Blood Detected and Quantified
2.0 (0.0-4.0) uM Adult (>18 years old) Both Normal
Cerebrospinal Fluid (CSF) Detected and Quantified
1 uM Children (1-13 year old) Both Normal
Cerebrospinal Fluid (CSF) Detected and Quantified
33.0 (9.00-57.0) uM Adult (>18 years old) Both Normal
Urine Detected and Quantified
1.0 (0.0-2.0) umol/mmol creatinine Adult (>18 years old) Both Normal
Urine Detected and Quantified
0.7 (0.2-1.7) umol/mmol creatinine Adult (>18 years old) Both Comment Normal
Urine Detected and Quantified
0.4 (0.2-0.8) umol/mmol creatinine Adult (>18 years old) Both Normal
Urine Detected and Quantified
0.1-0.4 umol/mmol creatinine Adult (>18 years old) Both Normal
Urine Detected and Quantified
0.1 (0.1-1.7) umol/mmol creatinine Children (1-13 year old) Both Normal
Urine Detected and Quantified
0.2 umol/mmol creatinine Adult (>18 years old) Both Normal
Urine Detected and Quantified
0.95 +/- 0.93 umol/mmol creatinine Adult (>18 years old) Both Normal
Urine Detected and Quantified
10.4 (2.8-53.7) umol/mmol creatinine Newborn (0-30 days old) Both Normal
Urine Detected and Quantified
0.5 (0.1-1.7) umol/mmol creatinine Adolescent (13-18 years old) Both Normal
Urine Detected and Quantified
10.7 (0.1-28.2) umol/mmol creatinine Infant (0-1 year old) Both Normal
Urine Detected but not Quantified
Not Applicable Adult (>18 years old) Male Normal
Abnormal Concentrations
Biofluid Status Value Age Sex Condition Reference
Blood Detected and Quantified 6.0 (4.0-8.0) uM Children (1-13 year old) Both Fumaric Aciduria
Cerebrospinal Fluid (CSF) Detected and Quantified 11.0 (2.00-20.0) uM Elderly (>65 years old) Both Alzheimer's disease
Urine Detected and Quantified 3500.0 (3000.0-4000.0) umol/mmol creatinine Children (1-13 year old) Both Fumaric Aciduria
Urine Detected and Quantified 0.7 (0.0-4.0) umol/mmol creatinine Adult (>18 years old) Both Lung cancer
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
      • Wishart DS, Knox C, Guo AC, Eisner R, Young N, Gautam B, Hau DD, Psychogios N, Dong E, Bouatra S, Mandal R, Sinelnikov I, Xia J, Jia L, Cruz JA, Lim E, Sobsey CA, Shrivastava S, Huang P, Liu P, Fang L, Peng J, Fradette R, Cheng D, Tzur D, Clements M, Lewis A, De Souza A, Zuniga A, Dawe M, Xiong Y, Clive D, Greiner R, Nazyrova A, Shaykhutdinov R, Li L, Vogel HJ, Forsythe I: HMDB: a knowledgebase for the human metabolome. Nucleic Acids Res. 2008 Oct 25. Pubmed: 18953024 Link_out
          Fumarase deficiency
              Associated OMIM IDs
              DrugBank ID DB01677 Link_out
              DrugBank Metabolite ID Not Available
              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:
              Ubiquinone-2 + Succinic acid unknown QH2 + Fumaric acid details
              Gene Name: SDHC
              Uniprot ID: Q99643 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial
              Reactions:
              Succinic acid + Coenzyme Q10 unknown Fumaric acid + QH(2) details
              Ubiquinone-2 + Succinic acid unknown QH2 + Fumaric acid details
              Gene Name: SDHA
              Uniprot ID: P31040 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial
              Reactions:
              Ubiquinone-2 + Succinic acid unknown QH2 + Fumaric acid details
              Gene Name: SDHD
              Uniprot ID: O14521 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial
              Reactions:
              Succinic acid + Coenzyme Q10 unknown Fumaric acid + QH(2) details
              Ubiquinone-2 + Succinic acid unknown QH2 + Fumaric acid details
              Gene Name: SDHB
              Uniprot ID: P21912 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Dopamine beta-hydroxylase
              Reactions: Not Available
              Gene Name: DBH
              Uniprot ID: P09172 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Adenylosuccinate lyase
              Reactions:
              N(6)-(1,2-dicarboxyethyl)AMP unknown Fumaric acid + Adenosine monophosphate details
              SAICAR unknown Fumaric acid + AICAR details
              SAICAR unknown Fumaric acid + AICAR details
              Gene Name: ADSL
              Uniprot ID: P30566 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Argininosuccinate lyase
              Reactions:
              Argininosuccinic acid unknown Fumaric acid + L-Arginine details
              Gene Name: ASL
              Uniprot ID: P04424 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Fumarylacetoacetase
              Reactions:
              4-Fumarylacetoacetic acid + Water unknown Acetoacetic acid + Fumaric acid details
              Acetoacetic acid + Fumaric acid unknown 4-Fumarylacetoacetic acid + Water details
              Gene Name: FAH
              Uniprot ID: P16930 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Fumarate hydratase, mitochondrial
              Reactions:
              L-Malic acid unknown Fumaric acid + Water details
              Gene Name: FH
              Uniprot ID: P07954 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: NAD-dependent malic enzyme, mitochondrial
              Reactions: Not Available
              Gene Name: ME2
              Uniprot ID: P23368 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Fumarate hydratase
              Reactions: Not Available
              Gene Name: FH
              Uniprot ID: B1ANK7 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Acylpyruvase FAHD1, mitochondrial
              Reactions:
              3-Fumarylpyruvate + Water unknown Fumaric acid + Pyruvic acid details
              Gene Name: FAHD1
              Uniprot ID: Q6P587 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA