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Record Information
Version4.0
Creation Date2006-02-02 11:25:17 UTC
Update Date2017-09-27 08:24:08 UTC
HMDB IDHMDB0001844
Secondary Accession Numbers
  • HMDB01844
Metabolite Identification
Common NameMethylsuccinic acid
DescriptionMethylsuccinic acid is a normal metabolite found in human fluids. Increased urinary levels of Methylsuccinic acid (together with ethylmalonic acid) are the main biochemical measurable features in ethylmalonic encephalopathy (OMIM 602473 ), a rare metabolic disorder with an autosomal recessive mode of inheritance that is clinically characterized by neuromotor delay, hyperlactic acidemia, recurrent petechiae, orthostatic acrocyanosis, and chronic diarrhea. (PMID 12382164 ) T he underlying biochemical defect involves isoleucine catabolism. (PMID 9667231 ). It has been found decreases in the urine of animals under D-serine-induced nephrotoxicity (D-Serine causes selective necrosis of the proximal straight tubules in the rat kidney). (PMID 15596249 ).
Structure
Thumb
Synonyms
ValueSource
(S)-2-Hydroxy-2-methylsuccinateHMDB
(S)-2-Hydroxy-2-methylsuccinic acidHMDB
(S)-CitramalateHMDB
(S)-Citramalic acidHMDB
2-MethylbutanedioateHMDB
2-Methylbutanedioic acidHMDB
2-MethylsuccinateHMDB
2-Methylsuccinic acidHMDB
Methyl succinateHMDB
Methyl succinic acidHMDB
MethylbutanedioateHMDB
Methylbutanedioic acidHMDB
MethylsuccinateHMDB
MEZHMDB
PyrotartarateHMDB
Pyrotartaric acidHMDB
Methylsuccinic acid, (+-)-isomerMeSH
Chemical FormulaC5H8O4
Average Molecular Weight132.1146
Monoisotopic Molecular Weight132.042258744
IUPAC Name2-methylbutanedioic acid
Traditional Namemethylsuccinic acid
CAS Registry Number498-21-5
SMILES
CC(CC(O)=O)C(O)=O
InChI Identifier
InChI=1S/C5H8O4/c1-3(5(8)9)2-4(6)7/h3H,2H2,1H3,(H,6,7)(H,8,9)
InChI KeyWXUAQHNMJWJLTG-UHFFFAOYSA-N
Chemical Taxonomy
DescriptionThis compound belongs to the class of chemical entities known as methyl-branched fatty acids. These are fatty acids with an acyl chain that has a methyl branch. Usually, they are saturated and contain only one or more methyl group. However, branches other than methyl may be present.
KingdomChemical entities
Super ClassOrganic compounds
ClassLipids and lipid-like molecules
Sub ClassFatty Acyls
Direct ParentMethyl-branched fatty acids
Alternative Parents
Substituents
  • Methyl-branched fatty acid
  • 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
Disposition

Biological Location:

  Subcellular:

  Biofluid and excreta:

Source:

  Biological:

    Animal:

Route of exposure:

  Enteral:

Role

Indirect biological role:

Biological role:

Process

Naturally occurring process:

  Biological process:

    Chemical reaction:

    Biochemical process:

    Biochemical pathway:

Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting Point117.5 °CNot Available
Boiling PointNot AvailableNot Available
Water Solubility516.5 mg/mLHuman Metabolome Project
LogPNot AvailableNot Available
Predicted Properties
PropertyValueSource
Water Solubility103 g/LALOGPS
logP0.13ALOGPS
logP0.14ChemAxon
logS-0.11ALOGPS
pKa (Strongest Acidic)3.99ChemAxon
Physiological Charge-2ChemAxon
Hydrogen Acceptor Count4ChemAxon
Hydrogen Donor Count2ChemAxon
Polar Surface Area74.6 ŲChemAxon
Rotatable Bond Count3ChemAxon
Refractivity28.11 m³·mol⁻¹ChemAxon
Polarizability11.91 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-b99795b0e5069d573c2dView in MoNA
GC-MSGC-MS Spectrum - EI-B (Non-derivatized)splash10-0002-0920000000-c96774045667ea1804dfView in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-0002-0910000000-b99795b0e5069d573c2dView in MoNA
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positivesplash10-007c-9100000000-bb48993db83be2104b10View in MoNA
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (2 TMS) - 70eV, Positivesplash10-00du-9320000000-5f34c9f97642781028e4View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 10V, Positive (Annotated)splash10-014i-3900000000-4afd6f9cd1309a7a392bView in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 25V, Positive (Annotated)splash10-00kr-9600000000-e212a368f465b41cc1faView in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 40V, Positive (Annotated)splash10-0l0i-9200000000-c712b36382fde51f3ae5View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QTOF (UPLC Q-Tof Premier, Waters) , Negativesplash10-000i-9400000000-f709b1c905420a006038View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QTOF (UPLC Q-Tof Premier, Waters) 30V, Negativesplash10-0019-9400000000-40a1055d8102e323a077View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QTOF , negativesplash10-000i-9400000000-f709b1c905420a006038View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QTOF , negativesplash10-0019-9400000000-40a1055d8102e323a077View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-014i-5900000000-e50cb8068b9ea6aa88c8View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-014r-9100000000-86a85f9d829697c248ddView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-0006-9000000000-687b61805462d04b5dedView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-001r-5900000000-4fd3ea5da8eb91f1951dView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-0019-9500000000-ec3019482baef20b293dView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-00kf-9000000000-23ddfbfc967e6df1168eView in MoNA
MSMass Spectrum (Electron Ionization)splash10-0077-9000000000-1bd08b69cec7f9966cb2View in MoNA
1D NMR1H NMR SpectrumNot AvailableView in JSpectraViewer
1D NMR1H NMR SpectrumNot AvailableView in JSpectraViewer
1D NMR13C NMR SpectrumNot AvailableView in JSpectraViewer
2D NMR[1H,13C] 2D NMR SpectrumNot AvailableView in JSpectraViewer
Biological Properties
Cellular Locations
  • Cytoplasm (predicted from logP)
Biofluid Locations
  • Blood
  • Feces
  • Saliva
  • Urine
Tissue LocationNot Available
PathwaysNot Available
NameSMPDB/PathwhizKEGG
No entries found
Normal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
BloodExpected but not Quantified Not AvailableNot Available
Normal
    details
    FecesDetected but not Quantified Adult (>18 years old)Both
    Normal
    details
    FecesDetected but not Quantified Not SpecifiedNot Specified
    Normal
    details
    FecesDetected but not Quantified Adult (>18 years old)Both
    Normal
    details
    FecesDetected and Quantified81.520 +/- 46.853 uMNot SpecifiedNot Specified
    Normal
    details
    FecesDetected and Quantified151.384 +/- 209.666 uMNot SpecifiedNot Specified
    Normal
    details
    FecesDetected but not Quantified Not SpecifiedNot Specified
    Normal
    details
    SalivaDetected and Quantified8.04 +/- 0.4 uMAdult (>18 years old)BothNormal
      • Zerihun T. Dame, ...
    details
    UrineDetected but not Quantified Adult (>18 years old)MaleNormal details
    UrineDetected but not Quantified Adult (>18 years old)BothNormal details
    UrineDetected and Quantified1.56 +/- 1.12 umol/mmol creatinineAdult (>18 years old)BothNot Available details
    UrineDetected and Quantified4.899 +/- 3.586 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
    Normal
      • Mordechai, Hien, ...
    details
    UrineDetected and Quantified5.06 +/- 0.99 umol/mmol creatinineChildren (1-13 years old)BothNormal details
    UrineDetected and Quantified0.69-1.2 umol/mmol creatinineAdult (>18 years old)FemaleNormal details
    UrineDetected and Quantified2.3 (0.8-10.8) umol/mmol creatinineAdult (>18 years old)BothNormal details
    UrineDetected but not Quantified Adult (>18 years old)Both
    Normal
    details
    UrineDetected and Quantified2.7 umol/mmol creatinineNewborn (0-30 days old)BothNormal details
    UrineDetected and Quantified4.8 umol/mmol creatinineInfant (0-1 year old)BothNormal details
    UrineDetected and Quantified2.8 umol/mmol creatinineChildren (1-13 years old)BothNormal details
    UrineDetected and Quantified2.6 umol/mmol creatinineChildren (1-13 years old)BothNormal details
    UrineDetected and Quantified2 umol/mmol creatinineAdult (>18 years old)BothNormal details
    UrineDetected and Quantified<3.89 umol/mmol creatinineChildren (1 - 18 years old)Both
    Normal
      • BC Children's Hos...
    details
    UrineDetected and Quantified0.53-0.85 umol/mmol creatinineAdult (>18 years old)MaleNormal details
    UrineDetected and Quantified4.7 (1.1-6.2) umol/mmol creatinineAdult (>18 years old)Both
    Normal
    details
    UrineDetected and Quantified3.2 (0.7-9.9) umol/mmol creatinineAdult (>18 years old)Both
    Normal
    details
    UrineDetected and Quantified1.065 +/- 0.863 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
    Normal
      • Mordechai, Hien, ...
    details
    Abnormal Concentrations
    BiofluidStatusValueAgeSexConditionReferenceDetails
    FecesDetected but not Quantified Adult (>18 years old)Both
    Irritable bowel syndrome
    details
    FecesDetected but not Quantified Adult (>18 years old)Both
    Ulcerative colitis
    details
    FecesDetected but not Quantified Adult (>18 years old)BothColorectal Cancer details
    UrineDetected and Quantified6.467 +/- 5.597 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
    Eosinophilic esophagitis
      • Mordechai, Hien, ...
    details
    UrineDetected and Quantified40.00 (20.00-60.00) umol/mmol creatinineChildren (1-13 years old)BothShort chain acyl-CoA dehydrogenase deficiency (SCAD) details
    UrineDetected and Quantified2.81 +/- 0.57 umol/mmol creatinineChildren (1-13 years old)BothMalnutrition (type kwashiorkor and marasmus) details
    UrineDetected and Quantified1.9 (1.3-2.5) umol/mmol creatinineAdult (>18 years old)BothRefsum's disease details
    UrineDetected and Quantified1.5 (0.0-3.0) umol/mmol creatinineAdult (>18 years old)BothEthylmalonic encephalopathy details
    UrineDetected and Quantified40.0 (20.0-60.0) umol/mmol creatinineChildren (1-13 years old)BothEthylmalonic encephalopathy details
    UrineDetected and Quantified25.309 +/- 83.321 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
    Eosinophilic esophagitis
      • Mordechai, Hien, ...
    details
    UrineDetected and Quantified1.628 +/- 1.644 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
    Gastroesophageal reflux disease
      • Mordechai, Hien, ...
    details
    Associated Disorders and Diseases
    Disease References
    Short Chain Acyl-Coa Dehydrogenase Deficiency
      MetaGene
    Refsum's disease
    1. Greter J, Lindstedt S, Steen G: 2,6-Dimethyloctanedioic acid--a metabolite of phytanic acid in Refsum's disease. Clin Chem. 1983 Mar;29(3):434-7. [PubMed:6186413 ]
    Ethylmalonic encephalopathy
      MetaGene
    Associated OMIM IDs
    DrugBank IDNot Available
    DrugBank Metabolite IDNot Available
    Phenol Explorer Compound IDNot Available
    Phenol Explorer Metabolite IDNot Available
    FoodDB IDFDB022704
    KNApSAcK IDNot Available
    Chemspider ID9922
    KEGG Compound IDC08645
    BioCyc IDDIETHYL-2R3R-2-METHYL-3-HYDROXYSUCCIN
    BiGG IDNot Available
    Wikipedia LinkNot Available
    METLIN ID6344
    PubChem Compound10349
    PDB IDNot Available
    ChEBI ID29003
    References
    Synthesis ReferenceNot Available
    Material Safety Data Sheet (MSDS)Download (PDF)
    General References
    1. 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 ]
    2. 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 ]
    3. Grosso S, Mostardini R, Farnetani MA, Molinelli M, Berardi R, Dionisi-Vici C, Rizzo C, Morgese G, Balestri P: Ethylmalonic encephalopathy: further clinical and neuroradiological characterization. J Neurol. 2002 Oct;249(10):1446-50. [PubMed:12382164 ]
    4. Nowaczyk MJ, Lehotay DC, Platt BA, Fisher L, Tan R, Phillips H, Clarke JT: Ethylmalonic and methylsuccinic aciduria in ethylmalonic encephalopathy arise from abnormal isoleucine metabolism. Metabolism. 1998 Jul;47(7):836-9. [PubMed:9667231 ]
    5. Williams RE, Major H, Lock EA, Lenz EM, Wilson ID: D-Serine-induced nephrotoxicity: a HPLC-TOF/MS-based metabonomics approach. Toxicology. 2005 Feb 14;207(2):179-90. [PubMed:15596249 ]