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Record Information
Version4.0
Creation Date2005-11-16 15:48:42 UTC
Update Date2017-09-27 06:56:46 UTC
HMDB IDHMDB0000134
Secondary Accession Numbers
  • HMDB00134
Metabolite Identification
Common NameFumaric acid
DescriptionFumaric acid is a dicarboxylic acid. It is a precursor to L-malate in the Krebs tricarboxylic acid (TCA) cycle. It is formed by the oxidation of succinic acid by succinate dehydrogenase. Fumarate is converted by the enzyme fumarase to malate. Fumaric acid has recently been identified as an oncometabolite or an endogenous, cancer causing metabolite. High levels of this organic acid can be found in tumors or biofluids surrounding tumors. Its oncogenic action appears to due to its ability to inhibit prolyl hydroxylase-containing enzymes. In many tumours, oxygen availability becomes limited (hypoxia) very quickly due to rapid cell proliferation and limited blood vessel growth. The major regulator of the response to hypoxia is the HIF transcription factor (HIF-alpha). Under normal oxygen levels, protein levels of HIF-alpha are very low due to constant degradation, mediated by a series of post-translational modification events catalyzed by the prolyl hydroxylase domain-containing enzymes PHD1, 2 and 3, (also known as EglN2, 1 and 3) that hydroxylate HIF-alpha and lead to its degradation. All three of the PHD enzymes are inhibited by fumarate.
Structure
Thumb
Synonyms
ValueSource
(2E)-2-Butenedioic acidChEBI
(e)-2-Butenedioic acidChEBI
e297ChEBI
FumarsaeureChEBI
trans-1,2-Ethylenedicarboxylic acidChEBI
trans-But-2-enedioic acidChEBI
trans-Butenedioic acidChEBI
(2E)-2-ButenedioateGenerator
FumarateGenerator
(e)-2-ButenedioateGenerator
trans-1,2-EthylenedicarboxylateGenerator
trans-But-2-enedioateGenerator
trans-ButenedioateGenerator
(2E)-But-2-enedioateHMDB
(2E)-But-2-enedioic acidHMDB
2-(e)-ButenedioateHMDB
2-(e)-Butenedioic acidHMDB
AllomaleateHMDB
Allomaleic acidHMDB
BoletateHMDB
Boletic acidHMDB
FC 33HMDB
LichenateHMDB
Lichenic acidHMDB
Sodium fumarateHMDB
trans-2-ButenedioateHMDB
trans-2-Butenedioic acidHMDB
FuramagMeSH
Ammonium fumarateMeSH
Magnesium fumarateMeSH
Chemical FormulaC4H4O4
Average Molecular Weight116.0722
Monoisotopic Molecular Weight116.010958616
IUPAC Name(2E)-but-2-enedioic acid
Traditional Namefumaric 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
DescriptionThis compound belongs to the class of organic compounds known as dicarboxylic acids and derivatives. These are organic compounds containing exactly two carboxylic acid groups.
KingdomOrganic compounds
Super ClassOrganic acids and derivatives
ClassCarboxylic acids and derivatives
Sub ClassDicarboxylic acids and derivatives
Direct ParentDicarboxylic acids and derivatives
Alternative Parents
Substituents
  • Fatty acyl
  • Fatty acid
  • Unsaturated fatty acid
  • Dicarboxylic acid or derivatives
  • Carboxylic acid
  • Organic oxygen compound
  • Organic oxide
  • Hydrocarbon derivative
  • Organooxygen compound
  • Carbonyl group
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors
Ontology
Physiological effect

Health effect:

  Health condition:

    Cancer:

    Gastrointestinal disorders:

    Respiratory, thoracic and mediastinal disorders:

    Nervous system disorders:

    Metabolism and nutrition disorders:

Disposition

Biological Location:

  Subcellular:

  Biofluid and excreta:

  Organ and components:

Source:

Route of exposure:

  Enteral:

Process

Naturally occurring process:

  Biological process:

    Biochemical pathway:

    Cellular process:

Role

Industrial application:

  Pharmaceutical industry:

Indirect biological role:

Biological role:

Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting Point549 °CNot Available
Boiling PointNot AvailableNot Available
Water Solubility7 mg/mLNot Available
LogP0.46HANSCH,C ET AL. (1995)
Predicted Properties
PropertyValueSource
Water Solubility24.1 g/LALOGPS
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 ŲChemAxon
Rotatable Bond Count2ChemAxon
Refractivity24.61 m³·mol⁻¹ChemAxon
Polarizability9.35 ųChemAxon
Number of Rings0ChemAxon
Bioavailability1ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectra
Spectra
Spectrum TypeDescriptionSplash Key
GC-MSGC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (2 TMS)splash10-0002-2940000000-e988056514d4ce4acc27View in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (2 TMS)splash10-0002-2960000000-a5ebaf2bbade922838ecView in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (2 TMS)splash10-0002-2950000000-32afa4d45e0e72b174b4View in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (Non-derivatized)splash10-0002-0950000000-fe0f05c02c783d0b6f6bView in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (2 TMS)splash10-006t-9530000000-0fc03f31f09dc8dbf4c6View in MoNA
GC-MSGC-MS Spectrum - GC-MS (2 TMS)splash10-0002-3690000000-75089756992cdbe841e3View in MoNA
GC-MSGC-MS Spectrum - EI-B (Non-derivatized)splash10-0002-9100000000-2cf649749b42cc0c610cView in MoNA
GC-MSGC-MS Spectrum - EI-B (Non-derivatized)splash10-0007-0890000000-b1c35cd55deb81254f66View in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-0002-2940000000-e988056514d4ce4acc27View in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-0002-2960000000-a5ebaf2bbade922838ecView in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-0002-2950000000-32afa4d45e0e72b174b4View in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-0002-0950000000-fe0f05c02c783d0b6f6bView in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-006t-9530000000-0fc03f31f09dc8dbf4c6View in MoNA
GC-MSGC-MS Spectrum - GC-MS (Non-derivatized)splash10-0002-3690000000-75089756992cdbe841e3View in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-0002-0940000000-177fdb9168659029ffaaView in MoNA
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positivesplash10-01ba-9200000000-52f88e04bac0ff8cdf17View in MoNA
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (2 TMS) - 70eV, Positivesplash10-00di-8920000000-06da44f348d0fe0358b3View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 10V, Negative (Annotated)splash10-00di-9100000000-57f13cd433a6fe4bf0b3View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 25V, Negative (Annotated)splash10-0229-9600000000-cd9e2979d0bb1e2a62f2View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 40V, Negative (Annotated)splash10-03k9-8900000000-dc50dbf8a50872383d54View in MoNA
LC-MS/MSLC-MS/MS Spectrum - EI-B (HITACHI RMU-6L) , Positivesplash10-0002-9100000000-b47e534bc82a6ed36e7cView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-014j-9800000000-dbf34563376daeb2901fView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-00xs-9200000000-113bf08cdc77707ed3adView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-00fr-9000000000-f32d7ec65e8649bc2b0aView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-014i-2900000000-408d53a9fff7acc8ca23View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-014i-6900000000-f149e9e5a34e27dd4d4eView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-0fxt-9000000000-cae5f71dc27daaf17d9eView in MoNA
MSMass Spectrum (Electron Ionization)splash10-0092-9000000000-003dd2d9303272b2ebeaView in MoNA
1D NMR1H NMR SpectrumNot AvailableView in JSpectraViewer
1D NMR13C NMR SpectrumNot AvailableView in JSpectraViewer
1D NMR1H NMR SpectrumNot AvailableView in JSpectraViewer
1D NMR13C NMR SpectrumNot AvailableView in JSpectraViewer
1D NMR1H NMR SpectrumNot AvailableView in JSpectraViewer
1D NMR13C NMR SpectrumNot AvailableView in JSpectraViewer
2D NMR[1H,1H] 2D NMR SpectrumNot AvailableView in JSpectraViewer
2D NMR[1H,13C] 2D NMR SpectrumNot AvailableView in JSpectraViewer
Biological Properties
Cellular Locations
  • Extracellular
  • Membrane
  • Mitochondria
Biofluid Locations
  • Blood
  • Breast Milk
  • Cerebrospinal Fluid (CSF)
  • Feces
  • Saliva
  • Sweat
  • Urine
Tissue Location
  • Prostate
Pathways
NameSMPDB/PathwhizKEGG
2-ketoglutarate dehydrogenase complex deficiencyPw000525Pw000525 greyscalePw000525 simpleNot Available
AICA-RibosiduriaPw000082Pw000082 greyscalePw000082 simpleNot Available
Adenine phosphoribosyltransferase deficiency (APRT)Pw000511Pw000511 greyscalePw000511 simpleNot Available
Adenosine Deaminase DeficiencyPw000075Pw000075 greyscalePw000075 simpleNot Available
Adenylosuccinate Lyase DeficiencyPw000076Pw000076 greyscalePw000076 simpleNot Available
Displaying entries 1 - 5 of 65 in total
Normal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified1.5 (0.0-4.0) uMAdult (>18 years old)BothNormal details
Breast MilkDetected and Quantified2.4 +/- 1.5 uMAdult (>18 years old)Female
Normal
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 Quantified Not SpecifiedNot Specified
Normal
details
FecesDetected but not Quantified Adult (>18 years old)Both
Normal
details
FecesDetected but not Quantified Adult (>18 years old)Both
Normal
details
FecesDetected but not Quantified Adult (>18 years old)Both
Normal
details
FecesDetected but not Quantified Adult (>18 years old)Both
Normal
details
FecesDetected and Quantified84.430 +/- 50.830 uMNot SpecifiedNot Specified
Normal
details
FecesDetected but not Quantified Adult (>18 years old)Not SpecifiedNormal details
FecesDetected but not Quantified Not SpecifiedNot Specified
Normal
details
FecesDetected but not Quantified Adult (>18 years old)BothNormal details
FecesDetected but not Quantified Adult (>18 years old)BothNormal details
FecesDetected but not Quantified Infant (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 Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Not SpecifiedNormal details
SalivaDetected and Quantified1.95 +/- 0.66 uMAdult (>18 years old)BothNormal
    • Zerihun T. Dame, ...
details
SweatDetected and Quantified10-40 uMAdult (60 years old)Male
Normal
details
SweatDetected and Quantified10 uMAdult (40 years old)Male
Normal
details
SweatDetected but not Quantified Adult BothNormal details
UrineDetected and Quantified0.80-1.8 umol/mmol creatinineAdult (>18 years old)FemaleNormal 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 Quantified<10.25 umol/mmol creatinineChildren (1 - 18 years old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified0.1-0.4 umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified0.75-1.2 umol/mmol creatinineAdult (>18 years old)MaleNormal details
UrineDetected and Quantified0.1 (0.1-1.7) umol/mmol creatinineChildren (1-13 years old)BothNormal details
UrineDetected and Quantified1.342 +/- 1.317 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Normal
    • Mordechai, Hien, ...
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 but not Quantified Adult (>18 years old)BothColorectal cancer details
BloodDetected and Quantified2.0 (0.0-4.0) uMAdult (>18 years old)BothFumarase deficiency details
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 Quantified Adult (>18 years old)BothColorectal Cancer details
FecesDetected but not Quantified Adult (>18 years old)Femaleankylosing spondylitis details
FecesDetected but not Quantified Adult (>18 years old)Femalerheumatoid arthritis details
FecesDetected but not Quantified Adult (>18 years old)BothColorectal Cancer details
FecesDetected but not Quantified Adult (>18 years old)Both
Irritable bowel syndrome
details
FecesDetected but not Quantified Adult (>18 years old)Not Specifiedasymptomatic diverticulosis details
FecesDetected but not Quantified Adult (>18 years old)Not Specifiedsymptomatic uncomplicated diverticular disease details
UrineDetected and Quantified1.0 (0.0-2.0) umol/mmol creatinineAdult (>18 years old)BothFumarase deficiency details
UrineDetected and Quantified3500.0 (3000.0-4000.0) umol/mmol creatinineChildren (1-13 years old)BothFumaric Aciduria details
UrineDetected but not Quantified Adult (>18 years old)BothColorectal cancer details
UrineDetected and Quantified0.7 (0.0-4.0) umol/mmol creatinineAdult (>18 years old)BothLung cancer details
UrineDetected and Quantified11.39 +/- 28.464 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Eosinophilic esophagitis
    • Mordechai, Hien, ...
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
    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. 2009 Jan;37(Database issue):D603-10. doi: 10.1093/nar/gkn810. Epub 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
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. doi: 10.1038/nature07762. [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