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Record Information
Version3.6
Creation Date2005-11-16 15:48:42 UTC
Update Date2016-06-10 21:22:34 UTC
HMDB IDHMDB00622
Secondary Accession NumbersNone
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
2-EthylpropanedioateGenerator
2-EthylmalonateGenerator
a-CarboxybutyrateGenerator
a-Carboxybutyric acidGenerator
alpha-CarboxybutyrateGenerator
α-carboxybutyrateGenerator
α-carboxybutyric acidGenerator
Ethyl-malonic acidHMDB
EthylmalonateHMDB
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 KeyInChIKey=UKFXDFUAPNAMPJ-UHFFFAOYSA-N
Chemical Taxonomy
DescriptionThis compound belongs to the class of organic compounds known as branched fatty acids. These are fatty acids containing a branched chain.
KingdomOrganic compounds
Super ClassLipids and lipid-like molecules
ClassFatty Acyls
Sub ClassFatty acids and conjugates
Direct ParentBranched fatty acids
Alternative Parents
Substituents
  • Branched fatty acid
  • 1,3-dicarbonyl compound
  • Dicarboxylic acid or derivatives
  • Carboxylic acid
  • Carboxylic acid derivative
  • Hydrocarbon derivative
  • Organooxygen compound
  • Carbonyl group
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors
Ontology
StatusDetected and Quantified
Origin
  • Endogenous
  • Food
Biofunction
  • Cell signaling
  • Fuel and energy storage
  • Fuel or energy source
  • Membrane integrity/stability
Application
  • Nutrients
  • Stabilizers
  • Surfactants and Emulsifiers
Cellular locations
  • Extracellular
  • Membrane
Physical Properties
StateLiquid
Experimental Properties
PropertyValueReference
Melting Point112 - 114 °CNot Available
Boiling PointNot AvailableNot Available
Water Solubility712.0 mg/mLNot Available
LogPNot AvailableNot Available
Predicted Properties
PropertyValueSource
Water Solubility94.1 mg/mLALOGPS
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 Å2ChemAxon
Rotatable Bond Count3ChemAxon
Refractivity28.16 m3·mol-1ChemAxon
Polarizability11.98 Å3ChemAxon
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)splash10-0002-0910000000-ff684346010454e439ecView 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
MSMass Spectrum (Electron Ionization)splash10-0udr-9300000000-e531a154ddaf695ebe5bView in MoNA
1D NMR1H NMR SpectrumNot Available
2D NMR[1H,13C] 2D NMR SpectrumNot Available
Biological Properties
Cellular Locations
  • Extracellular
  • Membrane
Biofluid Locations
  • Blood
  • Cerebrospinal Fluid (CSF)
  • Urine
Tissue Location
  • Fibroblasts
  • Prostate
  • Skeletal Muscle
PathwaysNot Available
Normal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
BloodExpected but not QuantifiedNot ApplicableNot AvailableNot Available
Normal
  • Not Applicable
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 QuantifiedNot ApplicableAdult (>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 QuantifiedNot ApplicableAdult (>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. [10197568 ]
Malonyl-Coa decarboxylase deficiency
  1. MetaGene [Link]
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
NuGOwiki LinkHMDB00622
Metagene LinkHMDB00622
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. [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. [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. [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. [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. [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. [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. [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. [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. [16773466 ]