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Record Information
Version4.0
Creation Date2005-11-16 15:48:42 UTC
Update Date2017-10-23 18:07:45 UTC
HMDB IDHMDB0000622
Secondary Accession Numbers
  • HMDB00622
Metabolite Identification
Common NameEthylmalonic acid
DescriptionEthylmalonic acid is identified in the urine of patients with short-chain acyl-coenzyme A dehydrogenase deficiency, which is a fatty acid metabolism disorder.
Structure
Thumb
Synonyms
ValueSource
1,1-Propanedicarboxylic acidChEBI
2-Ethylmalonic acidChEBI
alpha-Carboxybutyric acidChEBI
1,1-PropanedicarboxylateGenerator
EthylmalonateGenerator
2-EthylmalonateGenerator
a-CarboxybutyrateGenerator
a-Carboxybutyric acidGenerator
alpha-CarboxybutyrateGenerator
α-carboxybutyrateGenerator
α-carboxybutyric acidGenerator
Ethyl-malonic acidHMDB
Chemical FormulaC5H8O4
Average Molecular Weight132.1146
Monoisotopic Molecular Weight132.042258744
IUPAC Name2-ethylpropanedioic acid
Traditional Nameethylmalonic acid
CAS Registry Number601-75-2
SMILES
CCC(C(O)=O)C(O)=O
InChI Identifier
InChI=1S/C5H8O4/c1-2-3(4(6)7)5(8)9/h3H,2H2,1H3,(H,6,7)(H,8,9)
InChI KeyUKFXDFUAPNAMPJ-UHFFFAOYSA-N
Chemical Taxonomy
DescriptionThis compound belongs to the class of chemical entities known as branched fatty acids. These are fatty acids containing a branched chain.
KingdomChemical entities
Super ClassOrganic compounds
ClassLipids and lipid-like molecules
Sub ClassFatty Acyls
Direct ParentBranched fatty acids
Alternative Parents
Substituents
  • Branched fatty acid
  • 1,3-dicarbonyl compound
  • Dicarboxylic acid or derivatives
  • Carboxylic acid
  • Carboxylic acid derivative
  • 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:

Disposition

Biological Location:

  Subcellular:

  Biofluid and excreta:

  Organ and components:

  Tissue and substructures:

  Cell and elements:

    Cell:

Source:

  Biological:

    Animal:

Route of exposure:

  Enteral:

Process

Naturally occurring process:

  Biological process:

    Cellular process:

    Biochemical pathway:

    Chemical reaction:

    Biochemical process:

Role

Indirect biological role:

Industrial application:

Biological role:

Physical Properties
StateLiquid
Experimental Properties
PropertyValueReference
Melting Point112 - 114 °CNot Available
Boiling PointNot AvailableNot Available
Water Solubility712 mg/mLNot Available
LogPNot AvailableNot Available
Predicted Properties
PropertyValueSource
Water Solubility94.1 g/LALOGPS
logP0.43ALOGPS
logP0.66ChemAxon
logS-0.15ALOGPS
pKa (Strongest Acidic)2.5ChemAxon
Physiological Charge-2ChemAxon
Hydrogen Acceptor Count4ChemAxon
Hydrogen Donor Count2ChemAxon
Polar Surface Area74.6 ŲChemAxon
Rotatable Bond Count3ChemAxon
Refractivity28.16 m³·mol⁻¹ChemAxon
Polarizability11.98 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) (Non-derivatized)splash10-0002-0910000000-ff684346010454e439ecView in MoNA
GC-MSGC-MS Spectrum - EI-B (Non-derivatized)splash10-0gvx-9100000000-9713da0bdd2cd0b28ebaView in MoNA
GC-MSGC-MS Spectrum - EI-B (Non-derivatized)splash10-0002-0920000000-c6908d036ed13e7ba519View in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-0002-0910000000-ff684346010454e439ecView in MoNA
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positivesplash10-004u-9400000000-c51992693b05221f3a8aView in MoNA
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (2 TMS) - 70eV, Positivesplash10-00dr-9220000000-10ce85512076eaa6afdeView in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 10V, Negative (Annotated)splash10-000l-9000000000-ed08eca90f357944aee9View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 25V, Negative (Annotated)splash10-0006-9000000000-b6e83c7aef56876fa1b3View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 40V, Negative (Annotated)splash10-0006-9000000000-409423c1bfecc553b24eView in MoNA
LC-MS/MSLC-MS/MS Spectrum - , negativesplash10-000i-9000000000-d15979561ae10b3c94dfView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-00lr-3900000000-7ad5e5da82f0646b39dbView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-014i-9600000000-00a7e2649b402719b2faView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-00kf-9000000000-6cd3362f5cdb53773ce8View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-0019-9400000000-257e80506d0172d05d98View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-000l-9000000000-e79d02ff9ead3516adaaView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-0006-9000000000-c55403c45b1243b9628eView in MoNA
MSMass Spectrum (Electron Ionization)splash10-0udr-9300000000-e531a154ddaf695ebe5bView in MoNA
1D NMR1H NMR SpectrumNot AvailableView in JSpectraViewer
2D NMR[1H,13C] 2D NMR SpectrumNot AvailableView in JSpectraViewer
Biological Properties
Cellular Locations
  • Extracellular
  • Membrane
Biofluid Locations
  • Blood
  • Cerebrospinal Fluid (CSF)
  • Urine
Tissue Location
  • Fibroblasts
  • Prostate
  • Skeletal Muscle
PathwaysNot Available
NameSMPDB/PathwhizKEGG
No entries found
Normal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
BloodExpected but not Quantified Not AvailableNot Available
Normal
    details
    Cerebrospinal Fluid (CSF)Detected and Quantified0.84(0.15-0.69) uMChildren (1-13 years old)BothNormal details
    UrineDetected and Quantified1.16-2.99 umol/mmol creatinineAdult (>18 years old)BothNormal details
    UrineDetected and Quantified1.3 +/- 2.8 umol/mmol creatinineAdolescent (13-18 years old)FemaleNormal details
    UrineDetected and Quantified2.053 +/- 1.16 umol/mmol creatinineAdult (>18 years old)BothNot Available details
    UrineDetected and Quantified5.5 (0.1-18.6) umol/mmol creatinineNewborn (0-30 days old)BothNormal details
    UrineDetected and Quantified3.3 (0.1-11.0) umol/mmol creatinineInfant (0-1 year old)BothNormal details
    UrineDetected and Quantified4.8 (0.1-17.9) umol/mmol creatinineChildren (1-13 years old)Both
    Normal
    details
    UrineDetected and Quantified2.9 (0.8-5.3) umol/mmol creatinineChildren (1-13 years old)Both
    Normal
    details
    UrineDetected and Quantified2.2 (0.2-6.6) umol/mmol creatinineAdolescent (13-18 years old)Both
    Normal
    details
    UrineDetected but not Quantified Adult (>18 years old)BothNormal details
    UrineDetected and Quantified3.64 +/- 0.80 umol/mmol creatinineChildren (1-13 years old)BothNormal details
    UrineDetected and Quantified0.14 +/- 0.27 umol/mmol creatinineInfant (0-1 year old)BothNormal details
    UrineDetected and Quantified1.9-3.1 umol/mmol creatinineAdult (>18 years old)FemaleNormal details
    UrineDetected but not Quantified Adult (>18 years old)Both
    Normal
    details
    UrineDetected and Quantified2.0 (0.5-3.95) umol/mmol creatinineAdult (>18 years old)BothNormal
      • Geigy Scientific ...
    details
    UrineDetected and Quantified20.0 (0.0-40.0) umol/mmol creatinineChildren (1-13 years old)BothNormal
      • Geigy Scientific ...
    details
    UrineDetected and Quantified2.5 (0.4-4.2) umol/mmol creatinineAdult (>18 years old)BothNormal details
    UrineDetected and Quantified<11.08 umol/mmol creatinineChildren (1 - 18 years old)Both
    Normal
      • BC Children's Hos...
    details
    UrineDetected and Quantified3.0 (1.2-5.8) umol/mmol creatinineAdult (>18 years old)Both
    Normal
    details
    UrineDetected and Quantified1.5-2.6 umol/mmol creatinineAdult (>18 years old)MaleNormal details
    UrineDetected and Quantified6.946 +/- 4.98 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
    Normal
      • Mordechai, Hien, ...
    details
    UrineDetected and Quantified197.916 +/- 168.118 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
    Normal
      • Mordechai, Hien, ...
    details
    UrineDetected and Quantified2.4 (0.9-4.0) umol/mmol creatinineAdult (>18 years old)Both
    Normal
    details
    Abnormal Concentrations
    BiofluidStatusValueAgeSexConditionReferenceDetails
    UrineDetected and Quantified7.1 +/- 4.4 umol/mmol creatinineAdult (>18 years old)FemaleAnorexia nervosa details
    UrineDetected and Quantified7.649 +/- 5.821 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
    Eosinophilic esophagitis
      • Mordechai, Hien, ...
    details
    UrineDetected and Quantified243.043 +/- 176.552 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
    Eosinophilic esophagitis
      • Mordechai, Hien, ...
    details
    UrineDetected and Quantified218.665 +/- 89.032 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
    Gastroesophageal reflux disease
      • Mordechai, Hien, ...
    details
    UrineDetected and Quantified665.00 (180.00-1150.00) umol/mmol creatinineChildren (1-13 years old)BothMalonyl-CoA decarboxylase deficiency details
    UrineDetected and Quantified11.76 +/- 4.21 umol/mmol creatinineChildren (1-13 years old)BothMalnutrition (type kwashiorkor and marasmus) details
    Associated Disorders and Diseases
    Disease References
    Anorexia nervosa
    1. Capo-chichi CD, Gueant JL, Lefebvre E, Bennani N, Lorentz E, Vidailhet C, Vidailhet M: Riboflavin and riboflavin-derived cofactors in adolescent girls with anorexia nervosa. Am J Clin Nutr. 1999 Apr;69(4):672-8. [PubMed:10197568 ]
    Malonyl-Coa decarboxylase deficiency
      MetaGene
    Associated OMIM IDs
    • 606788 (Anorexia nervosa)
    • 248360 (Malonyl-Coa decarboxylase deficiency)
    DrugBank IDNot Available
    DrugBank Metabolite IDNot Available
    Phenol Explorer Compound IDNot Available
    Phenol Explorer Metabolite IDNot Available
    FoodDB IDFDB022147
    KNApSAcK IDNot Available
    Chemspider ID11263
    KEGG Compound IDNot Available
    BioCyc IDNot Available
    BiGG IDNot Available
    Wikipedia LinkNot Available
    METLIN ID4180
    PubChem Compound11756
    PDB IDNot Available
    ChEBI ID741548
    References
    Synthesis ReferenceZakharova, T. V.; Ternovaya, T. V.; Pirkes, S. B.; Kostromina, N. A. Study of complexing in the neodymium-ethylmalonic acid system by a spectrographic method. Zhurnal Neorganicheskoi Khimii (1979), 24(7), 1827-31.
    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. 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. Tiranti V, D'Adamo P, Briem E, Ferrari G, Mineri R, Lamantea E, Mandel H, Balestri P, Garcia-Silva MT, Vollmer B, Rinaldo P, Hahn SH, Leonard J, Rahman S, Dionisi-Vici C, Garavaglia B, Gasparini P, Zeviani M: Ethylmalonic encephalopathy is caused by mutations in ETHE1, a gene encoding a mitochondrial matrix protein. Am J Hum Genet. 2004 Feb;74(2):239-52. Epub 2004 Jan 19. [PubMed:14732903 ]
    4. McGowan KA, Nyhan WL, Barshop BA, Naviaux RK, Yu A, Haas RH, Townsend JJ: The role of methionine in ethylmalonic encephalopathy with petechiae. Arch Neurol. 2004 Apr;61(4):570-4. [PubMed:15096407 ]
    5. Birkebaek NH, Simonsen H, Gregersen N: Hypoglycaemia and elevated urine ethylmalonic acid in a child homozygous for the short-chain acyl-CoA dehydrogenase 625G > A gene variation. Acta Paediatr. 2002;91(4):480-2. [PubMed:12061367 ]
    6. Tanaka K, Ramsdell HS, Baretz BH, Keefe MB, Kean EA, Johnson B: Identification of ethylmalonic acid in urine of two patients with the vomitting sickness of Jamaica. Clin Chim Acta. 1976 May 17;69(1):105-12. [PubMed:1269146 ]
    7. Bhala A, Willi SM, Rinaldo P, Bennett MJ, Schmidt-Sommerfeld E, Hale DE: Clinical and biochemical characterization of short-chain acyl-coenzyme A dehydrogenase deficiency. J Pediatr. 1995 Jun;126(6):910-5. [PubMed:7776094 ]
    8. Bok LA, Vreken P, Wijburg FA, Wanders RJ, Gregersen N, Corydon MJ, Waterham HR, Duran M: Short-chain Acyl-CoA dehydrogenase deficiency: studies in a large family adding to the complexity of the disorder. Pediatrics. 2003 Nov;112(5):1152-5. [PubMed:14595061 ]
    9. Barschak AG, Ferreira Gda C, Andre KR, Schuck PF, Viegas CM, Tonin A, Dutra Filho CS, Wyse AT, Wannmacher CM, Vargas CR, Wajner M: Inhibition of the electron transport chain and creatine kinase activity by ethylmalonic acid in human skeletal muscle. Metab Brain Dis. 2006 Mar;21(1):11-9. Epub 2006 Apr 28. [PubMed:16773466 ]