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Showing metabocard for 2-Ethylhydracrylic acid (HMDB0000396)

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Record Information
Version5.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2023-02-21 17:14:47 UTC
HMDB IDHMDB0000396
Secondary Accession Numbers
  • HMDB0002321
  • HMDB00396
  • HMDB02321
Metabolite Identification
Common Name2-Ethylhydracrylic acid
DescriptionIsolated excretion of 2-ethylhydracrylic acid (2-methylbutyrylglycine; 2-MBG) is the hallmark of short/branched-chain acyl-CoA dehydrogenase deficiency (SBCADD), a defect in the proximal pathway of L-isoleucine oxidation (PMID: 15615815 ). Normal human urine contains small amounts (less than 4 mg/g of creatinine) of 2-ethylhydracrylic acid. Urinary excretion of 2-ethylhydracrylic acid is variably increased in defects of isoleucine oxidation at distal steps in the catabolic pathway (3-oxoacyl-CoA thiolase deficiency and methylmalonyl-CoA mutase deficiency) and is diminished when proximal steps of the oxidative pathway are blocked as in branched-chain oxo acid decarboxylase deficiency (maple syrup urine disease) (PMID: 1016232 ). 2-Ethylhydracrylic acid has been identified in the human placenta (PMID: 32033212 ).
Structure
Data?1676999687
MOL3D MOLSDF3D SDFPDB3D PDBSMILESInChI

MOL for HMDB0000396 (2-Ethylhydracrylic acid)

  Mrv0541 02231218302D          

  8  7  0  0  0  0            999 V2000
   13.2458   -8.9254    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
   16.1037   -8.9254    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
   15.3893   -7.6879    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
   14.6748   -8.9254    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   13.9603   -8.5129    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   14.6748   -9.7504    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   13.9603  -10.1629    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   15.3893   -8.5129    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
  1  5  1  0  0  0  0
  2  8  1  0  0  0  0
  3  8  2  0  0  0  0
  4  5  1  0  0  0  0
  4  6  1  0  0  0  0
  4  8  1  0  0  0  0
  6  7  1  0  0  0  0
M  END
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3D MOL for HMDB0000396 (2-Ethylhydracrylic acid)

HMDB0000396
     RDKit          3D
2-Ethylhydracrylic acid
 18 17  0  0  0  0  0  0  0  0999 V2000
    2.1758   -0.0795    0.5608 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.0040   -0.6013   -0.2140 C   0  0  0  0  0  0  0  0  0  0  0  0
   -0.3187   -0.1737    0.3512 C   0  0  0  0  0  0  0  0  0  0  0  0
   -1.3983   -0.7845   -0.5467 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.6665   -0.4470   -0.1075 O   0  0  0  0  0  0  0  0  0  0  0  0
   -0.3988    1.3212    0.3233 C   0  0  0  0  0  0  0  0  0  0  0  0
   -0.5490    1.9917    1.3741 O   0  0  0  0  0  0  0  0  0  0  0  0
   -0.3073    2.0023   -0.8791 O   0  0  0  0  0  0  0  0  0  0  0  0
    2.9725   -0.8565    0.6066 H   0  0  0  0  0  0  0  0  0  0  0  0
    1.9198    0.2349    1.5905 H   0  0  0  0  0  0  0  0  0  0  0  0
    2.5739    0.8022    0.0010 H   0  0  0  0  0  0  0  0  0  0  0  0
    1.0240   -0.2936   -1.2838 H   0  0  0  0  0  0  0  0  0  0  0  0
    0.9944   -1.7269   -0.2431 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.5068   -0.5429    1.3693 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.2083   -0.4478   -1.5752 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.2104   -1.8874   -0.5140 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.1150   -1.2635    0.2184 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.9851    2.7522   -1.0318 H   0  0  0  0  0  0  0  0  0  0  0  0
  1  2  1  0
  2  3  1  0
  3  4  1  0
  4  5  1  0
  3  6  1  0
  6  7  2  0
  6  8  1  0
  1  9  1  0
  1 10  1  0
  1 11  1  0
  2 12  1  0
  2 13  1  0
  3 14  1  0
  4 15  1  0
  4 16  1  0
  5 17  1  0
  8 18  1  0
M  END
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3D SDF for HMDB0000396 (2-Ethylhydracrylic acid)

  Mrv0541 02231218302D          

  8  7  0  0  0  0            999 V2000
   13.2458   -8.9254    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
   16.1037   -8.9254    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
   15.3893   -7.6879    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
   14.6748   -8.9254    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   13.9603   -8.5129    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   14.6748   -9.7504    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   13.9603  -10.1629    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   15.3893   -8.5129    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
  1  5  1  0  0  0  0
  2  8  1  0  0  0  0
  3  8  2  0  0  0  0
  4  5  1  0  0  0  0
  4  6  1  0  0  0  0
  4  8  1  0  0  0  0
  6  7  1  0  0  0  0
M  END
> <DATABASE_ID>
HMDB0000396

> <DATABASE_NAME>
hmdb

> <SMILES>
CCC(CO)C(O)=O

> <INCHI_IDENTIFIER>
InChI=1S/C5H10O3/c1-2-4(3-6)5(7)8/h4,6H,2-3H2,1H3,(H,7,8)

> <INCHI_KEY>
ZMZQVAUJTDKQGE-UHFFFAOYSA-N

> <FORMULA>
C5H10O3

> <MOLECULAR_WEIGHT>
118.1311

> <EXACT_MASS>
118.062994186

> <JCHEM_ACCEPTOR_COUNT>
3

> <JCHEM_AVERAGE_POLARIZABILITY>
11.898270599053731

> <JCHEM_BIOAVAILABILITY>
1

> <JCHEM_DONOR_COUNT>
2

> <JCHEM_FORMAL_CHARGE>
0

> <JCHEM_GHOSE_FILTER>
0

> <JCHEM_IUPAC>
2-(hydroxymethyl)butanoic acid

> <ALOGPS_LOGP>
0.03

> <JCHEM_LOGP>
0.18397391933333335

> <ALOGPS_LOGS>
0.52

> <JCHEM_MDDR_LIKE_RULE>
0

> <JCHEM_NUMBER_OF_RINGS>
0

> <JCHEM_PHYSIOLOGICAL_CHARGE>
-1

> <JCHEM_PKA>
15.51028800103905

> <JCHEM_PKA_STRONGEST_ACIDIC>
4.5138551197178725

> <JCHEM_PKA_STRONGEST_BASIC>
-2.6964607220213894

> <JCHEM_POLAR_SURFACE_AREA>
57.53

> <JCHEM_REFRACTIVITY>
28.2208

> <JCHEM_ROTATABLE_BOND_COUNT>
3

> <JCHEM_RULE_OF_FIVE>
1

> <ALOGPS_SOLUBILITY>
3.87e+02 g/l

> <JCHEM_TRADITIONAL_IUPAC>
2-ethylhydracrylic acid

> <JCHEM_VEBER_RULE>
0

$$$$
Download

3D-SDF for HMDB0000396 (2-Ethylhydracrylic acid)

HMDB0000396
     RDKit          3D
2-Ethylhydracrylic acid
 18 17  0  0  0  0  0  0  0  0999 V2000
    2.1758   -0.0795    0.5608 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.0040   -0.6013   -0.2140 C   0  0  0  0  0  0  0  0  0  0  0  0
   -0.3187   -0.1737    0.3512 C   0  0  0  0  0  0  0  0  0  0  0  0
   -1.3983   -0.7845   -0.5467 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.6665   -0.4470   -0.1075 O   0  0  0  0  0  0  0  0  0  0  0  0
   -0.3988    1.3212    0.3233 C   0  0  0  0  0  0  0  0  0  0  0  0
   -0.5490    1.9917    1.3741 O   0  0  0  0  0  0  0  0  0  0  0  0
   -0.3073    2.0023   -0.8791 O   0  0  0  0  0  0  0  0  0  0  0  0
    2.9725   -0.8565    0.6066 H   0  0  0  0  0  0  0  0  0  0  0  0
    1.9198    0.2349    1.5905 H   0  0  0  0  0  0  0  0  0  0  0  0
    2.5739    0.8022    0.0010 H   0  0  0  0  0  0  0  0  0  0  0  0
    1.0240   -0.2936   -1.2838 H   0  0  0  0  0  0  0  0  0  0  0  0
    0.9944   -1.7269   -0.2431 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.5068   -0.5429    1.3693 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.2083   -0.4478   -1.5752 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.2104   -1.8874   -0.5140 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.1150   -1.2635    0.2184 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.9851    2.7522   -1.0318 H   0  0  0  0  0  0  0  0  0  0  0  0
  1  2  1  0
  2  3  1  0
  3  4  1  0
  4  5  1  0
  3  6  1  0
  6  7  2  0
  6  8  1  0
  1  9  1  0
  1 10  1  0
  1 11  1  0
  2 12  1  0
  2 13  1  0
  3 14  1  0
  4 15  1  0
  4 16  1  0
  5 17  1  0
  8 18  1  0
M  END
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PDB for HMDB0000396 (2-Ethylhydracrylic acid)

HEADER    PROTEIN                                 23-FEB-12   NONE
TITLE     NULL                                                        
COMPND    NULL                                                        
SOURCE    NULL                                                        
KEYWDS    NULL                                                        
EXPDTA    NULL                                                        
AUTHOR    Marvin                                                      
REVDAT   1   23-FEB-12         0                                  
HETATM    1  O   UNK     0      24.725 -16.661   0.000  0.00  0.00           O+0
HETATM    2  O   UNK     0      30.060 -16.661   0.000  0.00  0.00           O+0
HETATM    3  O   UNK     0      28.727 -14.351   0.000  0.00  0.00           O+0
HETATM    4  C   UNK     0      27.393 -16.661   0.000  0.00  0.00           C+0
HETATM    5  C   UNK     0      26.059 -15.891   0.000  0.00  0.00           C+0
HETATM    6  C   UNK     0      27.393 -18.201   0.000  0.00  0.00           C+0
HETATM    7  C   UNK     0      26.059 -18.971   0.000  0.00  0.00           C+0
HETATM    8  C   UNK     0      28.727 -15.891   0.000  0.00  0.00           C+0
CONECT    1    5                                                            
CONECT    2    8                                                            
CONECT    3    8                                                            
CONECT    4    5    6    8                                                  
CONECT    5    1    4                                                       
CONECT    6    4    7                                                       
CONECT    7    6                                                            
CONECT    8    2    3    4                                                  
MASTER        0    0    0    0    0    0    0    0    8    0   14    0
END
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3D PDB for HMDB0000396 (2-Ethylhydracrylic acid)

COMPND    HMDB0000396
HETATM    1  C1  UNL     1       2.176  -0.079   0.561  1.00  0.00           C  
HETATM    2  C2  UNL     1       1.004  -0.601  -0.214  1.00  0.00           C  
HETATM    3  C3  UNL     1      -0.319  -0.174   0.351  1.00  0.00           C  
HETATM    4  C4  UNL     1      -1.398  -0.785  -0.547  1.00  0.00           C  
HETATM    5  O1  UNL     1      -2.667  -0.447  -0.108  1.00  0.00           O  
HETATM    6  C5  UNL     1      -0.399   1.321   0.323  1.00  0.00           C  
HETATM    7  O2  UNL     1      -0.549   1.992   1.374  1.00  0.00           O  
HETATM    8  O3  UNL     1      -0.307   2.002  -0.879  1.00  0.00           O  
HETATM    9  H1  UNL     1       2.973  -0.857   0.607  1.00  0.00           H  
HETATM   10  H2  UNL     1       1.920   0.235   1.590  1.00  0.00           H  
HETATM   11  H3  UNL     1       2.574   0.802   0.001  1.00  0.00           H  
HETATM   12  H4  UNL     1       1.024  -0.294  -1.284  1.00  0.00           H  
HETATM   13  H5  UNL     1       0.994  -1.727  -0.243  1.00  0.00           H  
HETATM   14  H6  UNL     1      -0.507  -0.543   1.369  1.00  0.00           H  
HETATM   15  H7  UNL     1      -1.208  -0.448  -1.575  1.00  0.00           H  
HETATM   16  H8  UNL     1      -1.210  -1.887  -0.514  1.00  0.00           H  
HETATM   17  H9  UNL     1      -3.115  -1.264   0.218  1.00  0.00           H  
HETATM   18  H10 UNL     1      -0.985   2.752  -1.032  1.00  0.00           H  
CONECT    1    2    9   10   11
CONECT    2    3   12   13
CONECT    3    4    6   14
CONECT    4    5   15   16
CONECT    5   17
CONECT    6    7    7    8
CONECT    8   18
END
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SMILES for HMDB0000396 (2-Ethylhydracrylic acid)

CCC(CO)C(O)=O
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INCHI for HMDB0000396 (2-Ethylhydracrylic acid)

InChI=1S/C5H10O3/c1-2-4(3-6)5(7)8/h4,6H,2-3H2,1H3,(H,7,8)
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View in JSmolView Stereo Labels
Synonyms
Chemical FormulaC5H10O3
Average Molecular Weight118.1311
Monoisotopic Molecular Weight118.062994186
IUPAC Name2-(hydroxymethyl)butanoic acid
Traditional Name2-ethylhydracrylic acid
CAS Registry Number4374-62-3
SMILES
CCC(CO)C(O)=O
InChI Identifier
InChI=1S/C5H10O3/c1-2-4(3-6)5(7)8/h4,6H,2-3H2,1H3,(H,7,8)
InChI KeyZMZQVAUJTDKQGE-UHFFFAOYSA-N
Chemical Taxonomy
Description Belongs to the class of organic compounds known as hydroxy fatty acids. These are fatty acids in which the chain bears a hydroxyl group.
KingdomOrganic compounds
Super ClassLipids and lipid-like molecules
ClassFatty Acyls
Sub ClassFatty acids and conjugates
Direct ParentHydroxy fatty acids
Alternative Parents
Substituents
  • Hydroxy fatty acid
  • Branched fatty acid
  • Beta-hydroxy acid
  • Hydroxy acid
  • Monocarboxylic acid or derivatives
  • Carboxylic acid
  • Carboxylic acid derivative
  • Organic oxygen compound
  • Organic oxide
  • Hydrocarbon derivative
  • Primary alcohol
  • Organooxygen compound
  • Carbonyl group
  • Alcohol
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors
Ontology
Physiological effectNot Available
Disposition
Biological locationRoute of exposureSource
Process
Naturally occurring process
Role
Biological roleIndustrial application
Physical Properties
StateSolid
Experimental Molecular Properties
PropertyValueReference
Melting PointNot AvailableNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Experimental Chromatographic PropertiesNot Available
Predicted Molecular Properties
Predicted Chromatographic Properties
Spectra
Biological Properties
Cellular LocationsNot Available
Biospecimen Locations
  • Blood
  • Feces
  • Urine
Tissue Locations
  • Placenta
Pathways
Normal Concentrations
Abnormal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
UrineDetected and Quantified3700 umol/mmol creatinineChildren (1-13 years old)Male3-Methylglutaconic Aciduria (Type V) details
UrineDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothBladder cancer details
UrineDetected and Quantified4-31 umol/mmol creatinineChildren (1-13 years old)Both2-Methylbutyryl-CoA dehydrogenase deficiency (SBACDD) details
UrineDetected and Quantified1.914-30.626 umol/mmol creatinineChildren (1-13 years old)Not Specified3-methylglutaconic aciduria type II, X-linked details
Associated Disorders and Diseases
Disease References
3-methylglutaconic aciduria type II, X-linked
  1. Kelley RI, Cheatham JP, Clark BJ, Nigro MA, Powell BR, Sherwood GW, Sladky JT, Swisher WP: X-linked dilated cardiomyopathy with neutropenia, growth retardation, and 3-methylglutaconic aciduria. J Pediatr. 1991 Nov;119(5):738-47. [PubMed:1719174 ]
Short/branched chain acyl-CoA dehydrogenase deficiency
  1. Sass JO, Ensenauer R, Roschinger W, Reich H, Steuerwald U, Schirrmacher O, Engel K, Haberle J, Andresen BS, Megarbane A, Lehnert W, Zschocke J: 2-Methylbutyryl-coenzyme A dehydrogenase deficiency: functional and molecular studies on a defect in isoleucine catabolism. Mol Genet Metab. 2008 Jan;93(1):30-5. doi: 10.1016/j.ymgme.2007.09.002. Epub 2007 Oct 22. [PubMed:17945527 ]
3-Methylglutaconic Aciduria type V
  1. Ojala T, Polinati P, Manninen T, Hiippala A, Rajantie J, Karikoski R, Suomalainen A, Tyni T: New mutation of mitochondrial DNAJC19 causing dilated and noncompaction cardiomyopathy, anemia, ataxia, and male genital anomalies. Pediatr Res. 2012 Oct;72(4):432-7. doi: 10.1038/pr.2012.92. Epub 2012 Jul 13. [PubMed:22797137 ]
Associated OMIM IDs
  • 302060 (3-methylglutaconic aciduria type II, X-linked)
  • 610006 (Short/branched chain acyl-CoA dehydrogenase deficiency)
  • 610198 (3-Methylglutaconic Aciduria type V)
DrugBank IDNot Available
Phenol Explorer Compound IDNot Available
FooDB IDFDB022014
KNApSAcK IDNot Available
Chemspider ID164207
KEGG Compound IDNot Available
BioCyc IDNot Available
BiGG IDNot Available
Wikipedia LinkNot Available
METLIN ID3785
PubChem Compound188979
PDB IDNot Available
ChEBI ID82956
Food Biomarker OntologyNot Available
VMH IDNot Available
MarkerDB IDMDB00000156
Good Scents IDNot Available
References
Synthesis ReferencePrice, Charles C.; Cypher, George A.; Krishnamurti, I. V. Thermal reaction of formaldehyde with allyl cyanide. Journal of the American Chemical Society (1952), 74 2987-91.
Material Safety Data Sheet (MSDS)Not Available
General References
  1. Korman SH, Andresen BS, Zeharia A, Gutman A, Boneh A, Pitt JJ: 2-ethylhydracrylic aciduria in short/branched-chain acyl-CoA dehydrogenase deficiency: application to diagnosis and implications for the R-pathway of isoleucine oxidation. Clin Chem. 2005 Mar;51(3):610-7. Epub 2004 Dec 22. [PubMed:15615815 ]
  2. 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 ]
  3. Ohdoi C, Nyhan WL, Kuhara T: Chemical diagnosis of Lesch-Nyhan syndrome using gas chromatography-mass spectrometry detection. J Chromatogr B Analyt Technol Biomed Life Sci. 2003 Jul 15;792(1):123-30. [PubMed:12829005 ]
  4. Mamer OA, Tjoa SS, Scriver CR, Klassen GA: Demonstration of a new mammalian isoleucine catabolic pathway yielding an Rseries of metabolites. Biochem J. 1976 Dec 15;160(3):417-26. [PubMed:1016232 ]
  5. Elshenawy S, Pinney SE, Stuart T, Doulias PT, Zura G, Parry S, Elovitz MA, Bennett MJ, Bansal A, Strauss JF 3rd, Ischiropoulos H, Simmons RA: The Metabolomic Signature of the Placenta in Spontaneous Preterm Birth. Int J Mol Sci. 2020 Feb 4;21(3). pii: ijms21031043. doi: 10.3390/ijms21031043. [PubMed:32033212 ]

Enzymes

Enzyme Details
1. Sulfotransferase family cytosolic 2B member 1
General function:
Involved in sulfotransferase activity
Specific function:
Sulfotransferase that utilizes 3'-phospho-5'-adenylyl sulfate (PAPS) as sulfonate donor to catalyze the sulfate conjugation of many hormones, neurotransmitters, drugs and xenobiotic compounds. Sulfonation increases the water solubility of most compounds, and therefore their renal excretion, but it can also result in bioactivation to form active metabolites. Sulfates hydroxysteroids like DHEA. Isoform 1 preferentially sulfonates cholesterol, and isoform 2 avidly sulfonates pregnenolone but not cholesterol.
Gene Name:
SULT2B1
Uniprot ID:
O00204
Molecular weight:
39598.595
Reactions
2-Ethylhydracrylic acid → 2-[(sulfooxy)methyl]butanoic aciddetails
Enzyme Details
2. UDP-glucuronosyltransferase 1-1
General function:
Involved in transferase activity, transferring hexosyl groups
Specific function:
UDPGT is of major importance in the conjugation and subsequent elimination of potentially toxic xenobiotics and endogenous compounds. This isoform glucuronidates bilirubin IX-alpha to form both the IX-alpha-C8 and IX-alpha-C12 monoconjugates and diconjugate. Is also able to catalyze the glucuronidation of 17beta-estradiol, 17alpha-ethinylestradiol, 1-hydroxypyrene, 4-methylumbelliferone, 1-naphthol, paranitrophenol, scopoletin, and umbelliferone.
Gene Name:
UGT1A1
Uniprot ID:
P22309
Molecular weight:
59590.91
Reactions
2-Ethylhydracrylic acid → 6-(2-carboxy-2-ethylethoxy)-3,4,5-trihydroxyoxane-2-carboxylic aciddetails
2-Ethylhydracrylic acid → 3,4,5-trihydroxy-6-{[2-(hydroxymethyl)butanoyl]oxy}oxane-2-carboxylic aciddetails