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Identification Taxonomy Ontology Physical properties Spectra Biological properties Concentrations Links References enzymes (2) Show 2 proteins XML

Record Information

Version

5.0

Status

Expected but not Quantified

Creation Date

2006-05-22 14:17:34 UTC

Update Date

2020-09-15 18:51:49 UTC

HMDB ID

HMDB0002052

Secondary Accession Numbers

HMDB02052

Metabolite Identification

Common Name

Maleylacetoacetic acid

Description

Maleylacetoacetic acid, also known as 4-maleylacetoacetate, belongs to the class of organic compounds known as medium-chain keto acids and derivatives. These are keto acids with a 6 to 12 carbon atoms long side chain. Maleylacetoacetic acid is a very hydrophobic molecule, practically insoluble (in water), and relatively neutral. Maleylacetoacetic acid exists in all living organisms, ranging from bacteria to humans. Within humans, maleylacetoacetic acid participates in a number of enzymatic reactions. In particular, maleylacetoacetic acid can be converted into 4-fumarylacetoacetic acid through its interaction with the enzyme maleylacetoacetate isomerase. In addition, maleylacetoacetic acid can be biosynthesized from homogentisic acid; which is mediated by the enzyme homogentisate 1,2-dioxygenase. 4-Maleylacetoacetate (4-maleylacetoacetate) is an intermediate in the metabolism of tyrosine. In humans, maleylacetoacetic acid is involved in the metabolic disorder called tyrosinemia type I. Maleylacetoacetic acid is a potentially toxic compound.

Structure

MOL SDF 3D SDF PDB 3D PDB SMILES InChI


  Mrv1652309042000262D          

 14 13  0  0  0  0            999 V2000
10006.008110004.6587    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
10005.292610004.2472    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
10004.578210004.6587    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
10003.863510004.2472    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
10003.038110004.2472    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
10002.323510004.6587    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
10001.608810004.2472    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
10002.323510005.4841    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
10004.578210005.4841    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
10006.721510004.2472    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
10007.436910004.6587    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
10008.152510004.2472    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
10007.436910005.4841    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
10006.008110005.4841    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
  1  2  1  0  0  0  0
  1 10  1  0  0  0  0
  1 14  2  0  0  0  0
  2  3  1  0  0  0  0
  3  4  1  0  0  0  0
  3  9  2  0  0  0  0
  4  5  2  0  0  0  0
  5  6  1  0  0  0  0
  6  7  1  0  0  0  0
  6  8  2  0  0  0  0
 10 11  1  0  0  0  0
 11 12  1  0  0  0  0
 11 13  2  0  0  0  0
M  END
> <DATABASE_ID>
HMDB0002052

> <DATABASE_NAME>
hmdb

> <SMILES>
OC(=O)CC(=O)CC(=O)\C=C/C(O)=O

> <INCHI_IDENTIFIER>
InChI=1S/C8H8O6/c9-5(1-2-7(11)12)3-6(10)4-8(13)14/h1-2H,3-4H2,(H,11,12)(H,13,14)/b2-1-

> <INCHI_KEY>
GACSIVHAIFQKTC-UPHRSURJSA-N

> <FORMULA>
C8H8O6

> <MOLECULAR_WEIGHT>
200.1455

> <EXACT_MASS>
200.032087988

> <JCHEM_ACCEPTOR_COUNT>
6

> <JCHEM_ATOM_COUNT>
22

> <JCHEM_AVERAGE_POLARIZABILITY>
16.75405859034148

> <JCHEM_BIOAVAILABILITY>
1

> <JCHEM_DONOR_COUNT>
2

> <JCHEM_FORMAL_CHARGE>
0

> <JCHEM_GHOSE_FILTER>
1

> <JCHEM_IUPAC>
(2Z)-4,6-dioxooct-2-enedioic acid

> <ALOGPS_LOGP>
-0.19

> <JCHEM_LOGP>
0.4027936060000003

> <ALOGPS_LOGS>
-2.08

> <JCHEM_MDDR_LIKE_RULE>
0

> <JCHEM_NUMBER_OF_RINGS>
0

> <JCHEM_PHYSIOLOGICAL_CHARGE>
-2

> <JCHEM_PKA>
3.8248983902309295

> <JCHEM_PKA_STRONGEST_ACIDIC>
2.716169602537572

> <JCHEM_PKA_STRONGEST_BASIC>
-7.0576341820437145

> <JCHEM_POLAR_SURFACE_AREA>
108.74000000000001

> <JCHEM_REFRACTIVITY>
44.397400000000005

> <JCHEM_ROTATABLE_BOND_COUNT>
6

> <JCHEM_RULE_OF_FIVE>
1

> <ALOGPS_SOLUBILITY>
1.65e+00 g/l

> <JCHEM_TRADITIONAL_IUPAC>
maleylacetoacetic acid

> <JCHEM_VEBER_RULE>
0

$$$$

HEADER    PROTEIN                                 04-SEP-20   NONE
TITLE     NULL                                                        
COMPND    NULL                                                        
SOURCE    NULL                                                        
KEYWDS    NULL                                                        
EXPDTA    NULL                                                        
AUTHOR    Marvin                                                      
REVDAT   1   04-SEP-20         0                                  
HETATM    1  C   UNK     0    8677.8828675.363   0.000  0.00  0.00           C+0
HETATM    2  C   UNK     0    8676.5468674.595   0.000  0.00  0.00           C+0
HETATM    3  C   UNK     0    8675.2138675.363   0.000  0.00  0.00           C+0
HETATM    4  C   UNK     0    8673.8798674.595   0.000  0.00  0.00           C+0
HETATM    5  C   UNK     0    8672.3388674.595   0.000  0.00  0.00           C+0
HETATM    6  C   UNK     0    8671.0048675.363   0.000  0.00  0.00           C+0
HETATM    7  O   UNK     0    8669.6708674.595   0.000  0.00  0.00           O+0
HETATM    8  O   UNK     0    8671.0048676.904   0.000  0.00  0.00           O+0
HETATM    9  O   UNK     0    8675.2138676.904   0.000  0.00  0.00           O+0
HETATM   10  C   UNK     0    8679.2138674.595   0.000  0.00  0.00           C+0
HETATM   11  C   UNK     0    8680.5498675.363   0.000  0.00  0.00           C+0
HETATM   12  O   UNK     0    8681.8858674.595   0.000  0.00  0.00           O+0
HETATM   13  O   UNK     0    8680.5498676.904   0.000  0.00  0.00           O+0
HETATM   14  O   UNK     0    8677.8828676.904   0.000  0.00  0.00           O+0
CONECT    1    2   10   14                                                  
CONECT    2    1    3                                                       
CONECT    3    2    4    9                                                  
CONECT    4    3    5                                                       
CONECT    5    4    6                                                       
CONECT    6    5    7    8                                                  
CONECT    7    6                                                            
CONECT    8    6                                                            
CONECT    9    3                                                            
CONECT   10    1   11                                                       
CONECT   11   10   12   13                                                  
CONECT   12   11                                                            
CONECT   13   11                                                            
CONECT   14    1                                                            
MASTER        0    0    0    0    0    0    0    0   14    0   26    0
END

COMPND    https://cactus.nci.nih.gov/chemical/structure/OC(=O)CC(=O)CC(=O)%5CC=C/C(O)=O... 
AUTHOR    GENERATED BY OPEN BABEL 3.1.0
HETATM    1  O   UNL     1       5.113   0.141  -0.206  1.00  0.00           O  
HETATM    2  C   UNL     1       3.789   0.281  -0.039  1.00  0.00           C  
HETATM    3  O   UNL     1       3.330   1.352   0.281  1.00  0.00           O  
HETATM    4  C   UNL     1       2.874  -0.898  -0.249  1.00  0.00           C  
HETATM    5  C   UNL     1       1.448  -0.478  -0.000  1.00  0.00           C  
HETATM    6  O   UNL     1       1.201   0.661   0.316  1.00  0.00           O  
HETATM    7  C   UNL     1       0.332  -1.479  -0.145  1.00  0.00           C  
HETATM    8  C   UNL     1      -0.985  -0.810   0.156  1.00  0.00           C  
HETATM    9  O   UNL     1      -1.014   0.363   0.459  1.00  0.00           O  
HETATM   10  C   UNL     1      -2.234  -1.581   0.083  1.00  0.00           C  
HETATM   11  C   UNL     1      -3.418  -0.960   0.239  1.00  0.00           C  
HETATM   12  C   UNL     1      -3.575   0.446  -0.181  1.00  0.00           C  
HETATM   13  O   UNL     1      -4.700   1.120   0.128  1.00  0.00           O  
HETATM   14  O   UNL     1      -2.690   0.996  -0.805  1.00  0.00           O  
HETATM   15  H   UNL     1       5.660   0.925  -0.060  1.00  0.00           H  
HETATM   16  H   UNL     1       2.973  -1.256  -1.274  1.00  0.00           H  
HETATM   17  H   UNL     1       3.144  -1.695   0.443  1.00  0.00           H  
HETATM   18  H   UNL     1       0.318  -1.863  -1.165  1.00  0.00           H  
HETATM   19  H   UNL     1       0.489  -2.302   0.551  1.00  0.00           H  
HETATM   20  H   UNL     1      -2.202  -2.645  -0.096  1.00  0.00           H  
HETATM   21  H   UNL     1      -4.253  -1.490   0.672  1.00  0.00           H  
HETATM   22  H   UNL     1      -4.757   2.039  -0.169  1.00  0.00           H  
CONECT    1    2   15                                                 
CONECT    2    1    3    3    4                                       
CONECT    3    2    2                                                 
CONECT    4    2    5   16   17                                       
CONECT    5    4    6    6    7                                       
CONECT    6    5    5                                                 
CONECT    7    5    8   18   19                                       
CONECT    8    7    9    9   10                                       
CONECT    9    8    8                                                 
CONECT   10    8   11   11   20                                       
CONECT   11   10   10   12   21                                       
CONECT   12   11   13   14   14                                       
CONECT   13   12   22                                                 
CONECT   14   12   12                                                 
CONECT   15    1                                                      
CONECT   16    4                                                      
CONECT   17    4                                                      
CONECT   18    7                                                      
CONECT   19    7                                                      
CONECT   20   10                                                      
CONECT   21   11                                                      
CONECT   22   13                                                      
MASTER        0    0    0    0    0    0    0    0   22    0   22    0
END

View in JSmol View Stereo Labels

Synonyms

Value	Source
4-Maleylacetoacetate	ChEBI
4-Maleylacetoacetic acid	Kegg
Maleylacetoacetate	Generator
(2Z)-4,6-Dioxo-2-octenedioic acid	HMDB
Maleylacetoacetic acid	HMDB

Chemical Formula

C₈H₈O₆

Average Molecular Weight

200.1455

Monoisotopic Molecular Weight

200.032087988

IUPAC Name

(2Z)-4,6-dioxooct-2-enedioic acid

Traditional Name

maleylacetoacetic acid

CAS Registry Number

5698-52-2

SMILES

OC(=O)CC(=O)CC(=O)\C=C/C(O)=O

InChI Identifier

InChI=1S/C8H8O6/c9-5(1-2-7(11)12)3-6(10)4-8(13)14/h1-2H,3-4H2,(H,11,12)(H,13,14)/b2-1-

InChI Key

GACSIVHAIFQKTC-UPHRSURJSA-N

Chemical Taxonomy

Description

Belongs to the class of organic compounds known as medium-chain keto acids and derivatives. These are keto acids with a 6 to 12 carbon atoms long side chain.

Kingdom

Organic compounds

Super Class

Organic acids and derivatives

Class

Keto acids and derivatives

Sub Class

Medium-chain keto acids and derivatives

Direct Parent

Medium-chain keto acids and derivatives

Alternative Parents

Substituents

Medium-chain keto acid
Beta-keto acid
1,3-diketone
Beta-hydroxy ketone
Dicarboxylic acid or derivatives
Unsaturated fatty acid
1,3-dicarbonyl compound
Fatty acyl
Enone
Acryloyl-group
Alpha,beta-unsaturated ketone
Ketone
Carboxylic acid
Carboxylic acid derivative
Organooxygen compound
Organic oxide
Organic oxygen compound
Carbonyl group
Hydrocarbon derivative
Aliphatic acyclic compound

Molecular Framework

Aliphatic acyclic compounds

External Descriptors

oxo dicarboxylic acid (CHEBI:47904 )
beta-diketone (CHEBI:47904 )
Dicarboxylic acids (LMFA01170114 )

Ontology

Disposition

Source:

Endogenous

Biological location:

Subcellular:

Cytoplasm

Process

Naturally occurring process:

Biological process:

Biochemical pathway:

Physical Properties

State

Solid

Experimental Molecular Properties

Property	Value	Reference
Melting Point	Not Available	Not Available
Boiling Point	Not Available	Not Available
Water Solubility	Not Available	Not Available
LogP	Not Available	Not Available

Predicted Molecular Properties

Property	Value	Source
Water Solubility	1.65 g/L	ALOGPS
logP	-0.19	ALOGPS
logP	0.4	ChemAxon
logS	-2.1	ALOGPS
pKa (Strongest Acidic)	2.72	ChemAxon
pKa (Strongest Basic)	-7.1	ChemAxon
Physiological Charge	-2	ChemAxon
Hydrogen Acceptor Count	6	ChemAxon
Hydrogen Donor Count	2	ChemAxon
Polar Surface Area	108.74 Å²	ChemAxon
Rotatable Bond Count	6	ChemAxon
Refractivity	44.4 m³·mol⁻¹	ChemAxon
Polarizability	16.75 Å³	ChemAxon
Number of Rings	0	ChemAxon
Bioavailability	Yes	ChemAxon
Rule of Five	Yes	ChemAxon
Ghose Filter	Yes	ChemAxon
Veber's Rule	No	ChemAxon
MDDR-like Rule	No	ChemAxon

Predicted Spectral Properties

Collision Cross Sections

Name	Adduct	Type	Data Source	Value	Reference

Retention Indices

Underivatized

Not Available

Derivatized

Derivative	Value	Reference
Maleylacetoacetic acid,1TMS,#1	1850.5513	https://arxiv.org/abs/1905.12712
Maleylacetoacetic acid,1TMS,#2	1810.4581	https://arxiv.org/abs/1905.12712
Maleylacetoacetic acid,1TMS,#3	2029.7045	https://arxiv.org/abs/1905.12712
Maleylacetoacetic acid,1TMS,#4	2001.6766	https://arxiv.org/abs/1905.12712
Maleylacetoacetic acid,1TMS,#5	2028.0798	https://arxiv.org/abs/1905.12712
Maleylacetoacetic acid,2TMS,#1	1885.1394	https://arxiv.org/abs/1905.12712
Maleylacetoacetic acid,2TMS,#2	2101.4338	https://arxiv.org/abs/1905.12712
Maleylacetoacetic acid,2TMS,#3	2062.961	https://arxiv.org/abs/1905.12712
Maleylacetoacetic acid,2TMS,#4	2097.107	https://arxiv.org/abs/1905.12712
Maleylacetoacetic acid,2TMS,#5	2114.4175	https://arxiv.org/abs/1905.12712
Maleylacetoacetic acid,2TMS,#6	2055.0017	https://arxiv.org/abs/1905.12712
Maleylacetoacetic acid,2TMS,#7	2105.9683	https://arxiv.org/abs/1905.12712
Maleylacetoacetic acid,2TMS,#8	2270.0234	https://arxiv.org/abs/1905.12712
Maleylacetoacetic acid,1TBDMS,#1	2091.242	https://arxiv.org/abs/1905.12712
Maleylacetoacetic acid,1TBDMS,#2	2060.936	https://arxiv.org/abs/1905.12712
Maleylacetoacetic acid,1TBDMS,#3	2267.619	https://arxiv.org/abs/1905.12712
Maleylacetoacetic acid,1TBDMS,#4	2235.8083	https://arxiv.org/abs/1905.12712
Maleylacetoacetic acid,1TBDMS,#5	2268.5889	https://arxiv.org/abs/1905.12712
Maleylacetoacetic acid,2TBDMS,#1	2345.8335	https://arxiv.org/abs/1905.12712
Maleylacetoacetic acid,2TBDMS,#2	2538.5564	https://arxiv.org/abs/1905.12712
Maleylacetoacetic acid,2TBDMS,#3	2498.4558	https://arxiv.org/abs/1905.12712
Maleylacetoacetic acid,2TBDMS,#4	2538.3145	https://arxiv.org/abs/1905.12712
Maleylacetoacetic acid,2TBDMS,#5	2590.765	https://arxiv.org/abs/1905.12712
Maleylacetoacetic acid,2TBDMS,#6	2515.339	https://arxiv.org/abs/1905.12712
Maleylacetoacetic acid,2TBDMS,#7	2555.9463	https://arxiv.org/abs/1905.12712
Maleylacetoacetic acid,2TBDMS,#8	2715.279	https://arxiv.org/abs/1905.12712

Spectra

GC-MS

Spectrum Type	Description	Splash Key	Deposition Date	View
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positive	splash10-0007-9300000000-7b2ae90eb353073cb652	2017-09-01	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (2 TMS) - 70eV, Positive	splash10-05bu-7092000000-254fb0c9b4683fd48ef1	2017-10-06	View Spectrum

LC-MS/MS

Spectrum Type	Description	Splash Key	Deposition Date	View
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 10V, Positive	splash10-0fsi-1920000000-f36f976fb4a0271e936b	2016-08-01	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 20V, Positive	splash10-000i-6900000000-692cbcc9f99aac913e42	2016-08-01	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 40V, Positive	splash10-00rj-9200000000-8e21be4180492b988f54	2016-08-01	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 10V, Negative	splash10-0a4j-1900000000-ea634f49698a94be6311	2016-08-03	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 20V, Negative	splash10-0a4i-5900000000-4d5261faac7c9dc29c36	2016-08-03	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 40V, Negative	splash10-0a4i-9500000000-17b0a81ad882f626ac2a	2016-08-03	View Spectrum

Biological Properties

Cellular Locations

Cytoplasm (predicted from logP)

Biospecimen Locations

Not Available

Tissue Locations

Not Available

Pathways

Name	SMPDB/Pathwhiz	KEGG
Tyrosine Metabolism
Phenylalanine and Tyrosine Metabolism
Phenylketonuria		Not Available
Tyrosinemia Type 2 (or Richner-Hanhart syndrome)		Not Available
Tyrosinemia Type 3 (TYRO3)		Not Available

Normal Concentrations

Not Available

Abnormal Concentrations

Not Available

Associated Disorders and Diseases

Disease References

None

Associated OMIM IDs

None

External Links

DrugBank ID

Not Available

Phenol Explorer Compound ID

Not Available

FooDB ID

KNApSAcK ID

Chemspider ID

KEGG Compound ID

BioCyc ID

4-MALEYL-ACETOACETATE

BiGG ID

1485279

Wikipedia Link

4-Maleylacetoacetic_acid

METLIN ID

6462

PubChem Compound

5280393

PDB ID

Not Available

ChEBI ID

47904

Food Biomarker Ontology

Not Available

VMH ID

4MLACAC

MarkerDB ID

Not Available

References

Synthesis Reference

Not Available

Material Safety Data Sheet (MSDS)

Not Available

General References

Kvittingen EA, Halvorsen S, Jellum E: Deficient fumarylacetoacetate fumarylhydrolase activity in lymphocytes and fibroblasts from patients with hereditary tyrosinemia. Pediatr Res. 1983 Jul;17(7):541-4. [PubMed:6622096 ]
Gray RG, Patrick AD, Preston FE, Whitfield MF: Acute hereditary tyrosinaemia type I: clinical, biochemical and haematological studies in twins. J Inherit Metab Dis. 1981;4(1):37-40. [PubMed:6785523 ]

Enzymes

Enzyme Details

1. Maleylacetoacetate isomerase

General function:: Involved in catalytic activity
Specific function:: Bifunctional enzyme showing minimal glutathione-conjugating activity with ethacrynic acid and 7-chloro-4-nitrobenz-2-oxa-1,3-diazole and maleylacetoacetate isomerase activity. Has also low glutathione peroxidase activity with T-butyl and cumene hydroperoxides. Is able to catalyze the glutathione dependent oxygenation of dichloroacetic acid to glyoxylic acid.
Gene Name:: GSTZ1
Uniprot ID:: O43708
Molecular weight:: 17895.68

Reactions

Maleylacetoacetic acid → 4-Fumarylacetoacetic acid	details

Enzyme Details

2. Homogentisate 1,2-dioxygenase

General function:: Involved in homogentisate 1,2-dioxygenase activity
Specific function:: Not Available
Gene Name:: HGD
Uniprot ID:: Q93099
Molecular weight:: 49963.41

Reactions

Homogentisic acid + Oxygen → Maleylacetoacetic acid	details

Showing metabocard for Maleylacetoacetic acid (HMDB0002052)

MOL for HMDB0002052 (Maleylacetoacetic acid)

3D MOL for HMDB0002052 (Maleylacetoacetic acid)

3D SDF for HMDB0002052 (Maleylacetoacetic acid)

3D-SDF for HMDB0002052 (Maleylacetoacetic acid)

PDB for HMDB0002052 (Maleylacetoacetic acid)

3D PDB for HMDB0002052 (Maleylacetoacetic acid)

SMILES for HMDB0002052 (Maleylacetoacetic acid)

INCHI for HMDB0002052 (Maleylacetoacetic acid)

Structure for HMDB0002052 (Maleylacetoacetic acid)

3D Structure for HMDB0002052 (Maleylacetoacetic acid)

Collision Cross Sections

Retention Indices

Underivatized

Derivatized

GC-MS

LC-MS/MS

Enzymes

Reactions

Reactions