| 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 |
Download:
MOL |
SDF |
SMILES |
InChI
Display:
2D Structure |
3D Structure
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| Synonyms |
- (2E)-But-2-enedioate
- (2E)-But-2-enedioic acid
- (E)-2-Butenedioate
- (E)-2-Butenedioic acid
- 2-(E)-Butenedioate
- 2-(E)-Butenedioic acid
- Allomaleate
- Allomaleic acid
- Boletate
- Boletic acid
- FC 33
- Fumarate
- Fumaric acid
- Lichenate
- Lichenic acid
- Sodium fumarate
- trans-1,2-Ethylenedicarboxylate
- trans-1,2-Ethylenedicarboxylic acid
- trans-2-Butenedioate
- trans-2-Butenedioic acid
- trans-Butenedioate
- 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 |
|
| 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 |
|
| 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
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| Tissue Location |
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| Pathways |
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| Normal Concentrations |
|
| Blood |
Detected and Quantified |
|
1.5 (0.0-4.0) uM |
Adult (>18 years old) |
Both |
Normal |
Not Available |
| Cerebrospinal Fluid (CSF) |
Detected and Quantified |
|
1 uM |
Children (1-13 year old) |
Both |
Normal |
Not Available |
| Cerebrospinal Fluid (CSF) |
Detected and Quantified |
|
33.0 (9.00-57.0) uM |
Adult (>18 years old) |
Both |
Normal |
Not Available |
| Urine |
Detected and Quantified |
|
1.0 (0.0-2.0) umol/mmol creatinine |
Adult (>18 years old) |
Both |
Normal |
Not Available |
| Urine |
Detected and Quantified |
|
0.7 (0.2-1.7) umol/mmol creatinine |
Adult (>18 years old) |
Both |
Normal |
urine by NMR
|
| Urine |
Detected and Quantified |
|
0.4 (0.2-0.8) umol/mmol creatinine |
Adult (>18 years old) |
Both |
Normal |
Not Available |
| Urine |
Detected and Quantified |
|
0.1-0.4 umol/mmol creatinine |
Adult (>18 years old) |
Both |
Normal |
Not Available |
| Urine |
Detected and Quantified |
|
0.1 (0.1-1.7) umol/mmol creatinine |
Children (1-13 year old) |
Both |
Normal |
Not Available |
| Urine |
Detected and Quantified |
|
0.2 umol/mmol creatinine |
Adult (>18 years old) |
Both |
Normal |
Not Available |
| Urine |
Detected and Quantified |
|
0.95 +/- 0.93 umol/mmol creatinine |
Adult (>18 years old) |
Both |
Normal |
Not Available |
| Urine |
Detected and Quantified |
|
10.4 (2.8-53.7) umol/mmol creatinine |
Newborn (0-30 days old) |
Both |
Normal |
Not Available |
| Urine |
Detected and Quantified |
|
0.5 (0.1-1.7) umol/mmol creatinine |
Adolescent (13-18 years old) |
Both |
Normal |
Not Available |
| Urine |
Detected and Quantified |
|
10.7 (0.1-28.2) umol/mmol creatinine |
Infant (0-1 year old) |
Both |
Normal |
Not Available |
| Urine |
Detected and not Quantified |
|
Not Applicable |
Adult (>18 years old) |
Male |
Normal |
Not Available |
|
| Abnormal Concentrations |
|
| Blood |
Detected and Quantified |
|
2.0 (0.0-4.0) uM |
Adult (>18 years old) |
Both |
Fumaric aciduria |
Not Available |
| Blood |
Detected and Quantified |
|
6.0 (4.0-8.0) uM |
Adult (>18 years old) |
Both |
Fumaric Aciduria |
Not Available |
| Cerebrospinal Fluid (CSF) |
Detected and Quantified |
|
11.0 (2.00-20.0) uM |
Elderly (>65 years old) |
Both |
Alzheimer's disease |
Not Available |
| Urine |
Detected and Quantified |
|
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
|
| Lung Cancer |
|
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| Fumarase deficiency |
- http://www.metagene.de/program/d.prg?mp=FUMARIC%20ACIDURIA
|
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| Associated OMIM IDs |
|
| External Links |
| DrugBank ID |
DB01677  |
| Phenol Explorer Compound ID |
Not Available |
| Phenol Explorer Metabolite ID |
Not Available |
| FoodDB ID |
FDB003291 |
| KNApSAcK ID |
C00001183  |
| Chemspider ID |
10197150  |
| KEGG Compound ID |
C00122  |
| BioCyc ID |
FUM  |
| BiGG ID |
33938  |
| Wikipedia Link |
Fumaric acid  |
| NuGOwiki Link |
HMDB00134  |
| Metagene Link |
HMDB00134  |
| METLIN ID |
3242  |
| PubChem Compound |
444972  |
| PDB ID |
FUM  |
| ChEBI ID |
18012  |
| 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 |
- 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
- 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
- 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
- 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
- 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
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| Enzymes |
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| Name: |
Dopamine beta-hydroxylase
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| Reactions: |
- 3,4-dihydroxyphenethylamine + ascorbate + O2 = noradrenaline + dehydroascorbate + H2O [RN:R02535]
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| Gene Name: |
DBH |
| Uniprot ID: |
P09172  |
| Protein Sequence: |
FASTA |
| Gene Sequence: |
FASTA |
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| Name: |
Adenylosuccinate lyase
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| 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]
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| Gene Name: |
ADSL |
| Uniprot ID: |
P30566  |
| Protein Sequence: |
FASTA |
| Gene Sequence: |
FASTA |
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| Name: |
Fumarylacetoacetase
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| Reactions: |
- 4-fumarylacetoacetate + H2O = acetoacetate + fumarate [RN:R01364]
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| Gene Name: |
FAH |
| Uniprot ID: |
P16930  |
| Protein Sequence: |
FASTA |
| Gene Sequence: |
FASTA |
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