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Record Information
Version3.6
Creation Date2005-11-16 15:48:42 UTC
Update Date2014-12-01 23:10:15 UTC
HMDB IDHMDB00134
Secondary Accession NumbersNone
Metabolite Identification
Common NameFumaric acid
DescriptionFumaric 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
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 FormulaC4H4O4
Average Molecular Weight116.0722
Monoisotopic Molecular Weight116.010958616
IUPAC Name(2E)-but-2-enedioic acid
Traditional Namemaleic acid
CAS Registry Number110-17-8
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 KeyVZCYOOQTPOCHFL-OWOJBTEDSA-N
Chemical Taxonomy
KingdomOrganic Compounds
Super ClassLipids
ClassFatty Acids and Conjugates
Sub ClassUnsaturated 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 ParentUnsaturated Fatty Acids
Ontology
StatusDetected 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
StateSolid
Experimental Properties
PropertyValueReference
Melting Point549 °CNot Available
Boiling PointNot AvailableNot Available
Water Solubility7.0 mg/mLNot Available
LogP0.46HANSCH,C ET AL. (1995)
Predicted Properties
PropertyValueSource
Water Solubility24.1ALOGPS
logP0.21ALOGPS
logP-0.041ChemAxon
logS-0.68ALOGPS
pKa (Strongest Acidic)3.55ChemAxon
Physiological Charge-2ChemAxon
Hydrogen Acceptor Count4ChemAxon
Hydrogen Donor Count2ChemAxon
Polar Surface Area74.6 Å2ChemAxon
Rotatable Bond Count2ChemAxon
Refractivity24.61 m3·mol-1ChemAxon
Polarizability9.35 Å3ChemAxon
Spectra
SpectraGC-MSMS/MSMS1D NMR2D NMR
Biological Properties
Cellular Locations
  • Extracellular
  • Membrane
  • Mitochondria
Biofluid Locations
  • Blood
  • Cerebrospinal Fluid (CSF)
  • Feces
  • Saliva
  • Urine
Tissue Location
  • Prostate
Pathways
NameSMPDB LinkKEGG Link
Arginine and Proline MetabolismSMP00020map00330
Aspartate MetabolismSMP00067map00250
Citric Acid CycleSMP00057map00020
Mitochondrial Electron Transport ChainSMP00355map00190
Phenylalanine and Tyrosine MetabolismSMP00008map00360
Tyrosine MetabolismSMP00006map00350
Urea CycleSMP00059map00330
Normal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified1.5 (0.0-4.0) uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified2.0 (0.0-4.0) uMAdult (>18 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified1 uMChildren (1-13 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified33.0 (9.00-57.0) uMAdult (>18 years old)BothNormal details
FecesDetected but not QuantifiedNot ApplicableNot SpecifiedNot Specified
Normal
details
FecesDetected but not QuantifiedNot ApplicableAdult (>18 years old)Both
Normal
details
FecesDetected but not QuantifiedNot ApplicableAdult (>18 years old)Both
Normal
details
FecesDetected but not QuantifiedNot ApplicableAdult (>18 years old)Both
Normal
details
FecesDetected and Quantified84.430 +/- 50.830 uMNot SpecifiedNot Specified
Normal
details
FecesDetected but not QuantifiedNot ApplicableNot SpecifiedNot Specified
Normal
details
FecesDetected but not QuantifiedNot ApplicableInfant (0-1 year old)Both
Normal
details
SalivaDetected and Quantified0.707 +/- 0.303 uMAdult (>18 years old)Not Specified
Normal
    • Sugimoto et al. (...
details
SalivaDetected and Quantified0.778 +/- 1.07 uMAdult (>18 years old)Not Specified
Normal
    • Sugimoto et al. (...
details
SalivaDetected and Quantified0.798 +/- 0.396 uMAdult (>18 years old)Not Specified
Normal
    • Sugimoto et al. (...
details
SalivaDetected and Quantified0.997 +/- 0.492 uMAdult (>18 years old)Not Specified
Normal
    • Sugimoto et al. (...
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Not SpecifiedNormal details
SalivaDetected and Quantified1.95 +/- 0.66 uMAdult (>18 years old)BothNormal
    • Dame, ZT. et al. ...
details
UrineDetected and Quantified1.0 (0.0-2.0) umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified0.7 (0.2-1.7) umol/mmol creatinineAdult (>18 years old)Both
Normal
details
UrineDetected and Quantified0.4 (0.2-0.8) umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified0.1-0.4 umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified0.1 (0.1-1.7) umol/mmol creatinineChildren (1-13 years old)BothNormal details
UrineDetected and Quantified0.2 umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified0.95 +/- 0.93 umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified10.4 (2.8-53.7) umol/mmol creatinineNewborn (0-30 days old)BothNormal details
UrineDetected and Quantified0.5 (0.1-1.7) umol/mmol creatinineAdolescent (13-18 years old)BothNormal details
UrineDetected and Quantified10.7 (0.1-28.2) umol/mmol creatinineInfant (0-1 year old)BothNormal details
Abnormal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified6.0 (4.0-8.0) uMChildren (1-13 years old)BothFumaric Aciduria details
Cerebrospinal Fluid (CSF)Detected and Quantified11.0 (2.00-20.0) uMElderly (>65 years old)BothAlzheimer's disease details
FecesDetected but not QuantifiedNot ApplicableAdult (>18 years old)Both
Irritable bowel syndrome
details
UrineDetected and Quantified3500.0 (3000.0-4000.0) umol/mmol creatinineChildren (1-13 years old)BothFumaric Aciduria details
UrineDetected and Quantified0.7 (0.0-4.0) umol/mmol creatinineAdult (>18 years old)BothLung cancer details
Associated Disorders and Diseases
Disease References
Alzheimer's disease
  1. 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
Fumarase deficiency
  1. MetaGene
Lung Cancer
  1. 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
Associated OMIM IDs
DrugBank IDDB01677
DrugBank Metabolite IDNot Available
Phenol Explorer Compound IDNot Available
Phenol Explorer Metabolite IDNot Available
FoodDB IDFDB003291
KNApSAcK IDC00001183
Chemspider ID10197150
KEGG Compound IDC00122
BioCyc IDFUM
BiGG ID33938
Wikipedia LinkFumaric acid
NuGOwiki LinkHMDB00134
Metagene LinkHMDB00134
METLIN ID3242
PubChem Compound444972
PDB IDFUM
ChEBI ID18012
References
Synthesis ReferenceDong, 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. 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
  2. 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
  3. 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
  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
  5. 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

Enzymes

General function:
Involved in succinate dehydrogenase activity
Specific function:
Membrane-anchoring subunit of succinate dehydrogenase (SDH) that is involved in complex II of the mitochondrial electron transport chain and is responsible for transferring electrons from succinate to ubiquinone (coenzyme Q).
Gene Name:
SDHC
Uniprot ID:
Q99643
Molecular weight:
16650.185
Reactions
Ubiquinone-2 + Succinic acid → QH2 + Fumaric aciddetails
General function:
Involved in electron carrier activity
Specific function:
Flavoprotein (FP) subunit of succinate dehydrogenase (SDH) that is involved in complex II of the mitochondrial electron transport chain and is responsible for transferring electrons from succinate to ubiquinone (coenzyme Q). Can act as a tumor suppressor.
Gene Name:
SDHA
Uniprot ID:
P31040
Molecular weight:
72690.975
Reactions
Succinic acid + Coenzyme Q10 → Fumaric acid + QH(2)details
Ubiquinone-2 + Succinic acid → QH2 + Fumaric aciddetails
General function:
Involved in iron ion binding
Specific function:
Membrane-anchoring subunit of succinate dehydrogenase (SDH) that is involved in complex II of the mitochondrial electron transport chain and is responsible for transferring electrons from succinate to ubiquinone (coenzyme Q) (By similarity).
Gene Name:
SDHD
Uniprot ID:
O14521
Molecular weight:
17042.82
Reactions
Ubiquinone-2 + Succinic acid → QH2 + Fumaric aciddetails
General function:
Involved in electron carrier activity
Specific function:
Iron-sulfur protein (IP) subunit of succinate dehydrogenase (SDH) that is involved in complex II of the mitochondrial electron transport chain and is responsible for transferring electrons from succinate to ubiquinone (coenzyme Q).
Gene Name:
SDHB
Uniprot ID:
P21912
Molecular weight:
31629.365
Reactions
Succinic acid + Coenzyme Q10 → Fumaric acid + QH(2)details
Ubiquinone-2 + Succinic acid → QH2 + Fumaric aciddetails
General function:
Involved in monooxygenase activity
Specific function:
Conversion of dopamine to noradrenaline.
Gene Name:
DBH
Uniprot ID:
P09172
Molecular weight:
69064.45
General function:
Involved in catalytic activity
Specific function:
Not Available
Gene Name:
ADSL
Uniprot ID:
P30566
Molecular weight:
54888.735
Reactions
N(6)-(1,2-dicarboxyethyl)AMP → Fumaric acid + Adenosine monophosphatedetails
SAICAR → Fumaric acid + AICARdetails
SAICAR → Fumaric acid + AICARdetails
General function:
Involved in argininosuccinate lyase activity
Specific function:
Not Available
Gene Name:
ASL
Uniprot ID:
P04424
Molecular weight:
51657.505
Reactions
Argininosuccinic acid → Fumaric acid + L-Argininedetails
General function:
Involved in fumarylacetoacetase activity
Specific function:
Not Available
Gene Name:
FAH
Uniprot ID:
P16930
Molecular weight:
46373.97
Reactions
4-Fumarylacetoacetic acid + Water → Acetoacetic acid + Fumaric aciddetails
Acetoacetic acid + Fumaric acid → 4-Fumarylacetoacetic acid + Waterdetails
References
  1. Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. Pubmed: 17139284
  2. Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. Pubmed: 17016423
  3. Berman HM, Westbrook J, Feng Z, Gilliland G, Bhat TN, Weissig H, Shindyalov IN, Bourne PE: The Protein Data Bank. Nucleic Acids Res. 2000 Jan 1;28(1):235-42. Pubmed: 10592235
General function:
Involved in fumarate hydratase activity
Specific function:
Also acts as a tumor suppressor.
Gene Name:
FH
Uniprot ID:
P07954
Molecular weight:
54636.585
Reactions
L-Malic acid → Fumaric acid + Waterdetails
General function:
Involved in oxidoreductase activity
Specific function:
Not Available
Gene Name:
ME2
Uniprot ID:
P23368
Molecular weight:
53585.73
References
  1. Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. Pubmed: 17139284
  2. Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. Pubmed: 17016423
General function:
Involved in fumarate hydratase activity
Specific function:
Not Available
Gene Name:
FH
Uniprot ID:
B1ANK7
Molecular weight:
54636.6
General function:
Involved in catalytic activity
Specific function:
Probable mitochondrial acylpyruvase which is able to hydrolyze acetylpyruvate and fumarylpyruvate in vitro.
Gene Name:
FAHD1
Uniprot ID:
Q6P587
Molecular weight:
27128.185
Reactions
3-Fumarylpyruvate + Water → Fumaric acid + Pyruvic aciddetails