Methylmalonic acid (CHEM003364)

IdentificationTaxonomyBiological PropertiesPhysical PropertiesToxicity ProfileSpectraConcentrationsLinksReferencesXML
Record Information
Version1.0
Creation Date2014-08-29 06:51:02 UTC
Update Date2026-04-03 00:38:26 UTC
Accession NumberCHEM003364
Identification
Common NameMethylmalonic acid
ClassSmall Molecule
DescriptionMethylmalonic acid is a malonic acid derivative which is a vital intermediate in the metabolism of fat and protein. Abnormalities in methylmalonic acid metabolism lead to methylmalonic aciduria. This metabolic disease is attributed to a block in the enzymatic conversion of methylmalonyl CoA to succinyl CoA.
Contaminant Sources
  • FooDB Chemicals
  • HMDB Contaminants - Urine
  • T3DB toxins
Contaminant Type
  • Animal Toxin
  • Food Toxin
  • Metabolite
  • Natural Compound
  • Organic Compound
Chemical Structure
Thumb
MOLSDFPDBSMILESInChI
Synonyms
ValueSource
1,1-Ethanedicarboxylic acidChEBI
2-Methylmalonic acidChEBI
alpha-Methylmalonic acidChEBI
Isosuccinic acidChEBI
1,1-EthanedicarboxylateGenerator
2-MethylmalonateGenerator
a-MethylmalonateGenerator
a-Methylmalonic acidGenerator
alpha-MethylmalonateGenerator
Α-methylmalonateGenerator
Α-methylmalonic acidGenerator
IsosuccinateGenerator
MethylmalonateGenerator
Methyl-malonateHMDB
Methyl-malonic acidHMDB
Methyl-propanedioateHMDB
Methyl-propanedioic acidHMDB
MethylpropanedioateHMDB
Methylpropanedioic acidHMDB
Acid, methylmalonicHMDB
Methylmalonic acidKEGG
Chemical FormulaC4H6O4
Average Molecular Mass118.088 g/mol
Monoisotopic Mass118.027 g/mol
CAS Registry Number516-05-2
IUPAC Name2-methylpropanedioic acid
Traditional Namemethylmalonic acid
SMILESCC(C(O)=O)C(O)=O
InChI IdentifierInChI=1S/C4H6O4/c1-2(3(5)6)4(7)8/h2H,1H3,(H,5,6)(H,7,8)
InChI KeyZIYVHBGGAOATLY-UHFFFAOYSA-N
Chemical Taxonomy
Description belongs to the class of organic compounds known as dicarboxylic acids and derivatives. These are organic compounds containing exactly two carboxylic acid groups.
KingdomOrganic compounds
Super ClassOrganic acids and derivatives
ClassCarboxylic acids and derivatives
Sub ClassDicarboxylic acids and derivatives
Direct ParentDicarboxylic acids and derivatives
Alternative Parents
Substituents
  • 1,3-dicarbonyl compound
  • Dicarboxylic acid or derivatives
  • Carboxylic acid
  • Organic oxygen compound
  • Organic oxide
  • Hydrocarbon derivative
  • Organooxygen compound
  • Carbonyl group
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors
Biological Properties
StatusDetected and Not Quantified
OriginEndogenous
Cellular Locations
  • Cytoplasm
  • Extracellular
Biofluid LocationsNot Available
Tissue Locations
  • Kidney
  • Liver
Pathways
NameSMPDB LinkKEGG Link
Propanoate MetabolismSMP00016 map00640
Valine, Leucine and Isoleucine DegradationSMP00032 map00280
Malonyl-coa decarboxylase deficiencySMP00502 Not Available
Malonic AciduriaSMP00198 Not Available
Methylmalonate Semialdehyde Dehydrogenase DeficiencySMP00384 Not Available
Methylmalonic AciduriaSMP00200 Not Available
Methylmalonic Aciduria Due to Cobalamin-Related DisordersSMP00201 Not Available
ApplicationsNot Available
Biological Roles
Chemical RolesNot Available
Physical Properties
StateSolid
AppearanceWhite powder.
Experimental Properties
PropertyValue
Melting Point135°C
Boiling PointNot Available
Solubility679.0 mg/mL
Predicted Properties
PropertyValueSource
Water Solubility149 g/LALOGPS
logP0.17ALOGPS
logP0.21ChemAxon
logS0.1ALOGPS
pKa (Strongest Acidic)2.48ChemAxon
Physiological Charge-2ChemAxon
Hydrogen Acceptor Count4ChemAxon
Hydrogen Donor Count2ChemAxon
Polar Surface Area74.6 ŲChemAxon
Rotatable Bond Count2ChemAxon
Refractivity23.56 m³·mol⁻¹ChemAxon
Polarizability10.06 ųChemAxon
Number of Rings0ChemAxon
Bioavailability1ChemAxon
Rule of FiveYesChemAxon
Ghose FilterNoChemAxon
Veber's RuleNoChemAxon
MDDR-like RuleNoChemAxon
Spectra
Spectra
Spectrum TypeDescriptionSplash KeyView
GC-MSGC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (2 TMS)splash10-0002-1910000000-cf95dd8a481761a28664Spectrum
GC-MSGC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (2 TMS)splash10-006t-8900000000-4d8795e7e4300cc7ceffSpectrum
GC-MSGC-MS Spectrum - GC-MS (2 TMS)splash10-00lr-5940000000-56b86ada220d335ce30aSpectrum
GC-MSGC-MS Spectrum - GC-MS (3 TMS)splash10-001i-9301000000-54e5247d046de566ac1eSpectrum
GC-MSGC-MS Spectrum - EI-B (Non-derivatized)splash10-004i-9000000000-b9ee59dddf90dfc7c461Spectrum
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-0002-1910000000-cf95dd8a481761a28664Spectrum
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-006t-8900000000-4d8795e7e4300cc7ceffSpectrum
GC-MSGC-MS Spectrum - GC-MS (Non-derivatized)splash10-001i-9301000000-54e5247d046de566ac1eSpectrum
GC-MSGC-MS Spectrum - GC-MS (Non-derivatized)splash10-00lr-5940000000-56b86ada220d335ce30aSpectrum
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-0002-0900000000-684a11ce99cccbd9194aSpectrum
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positivesplash10-0g4i-9400000000-baa4ea6d613ebb8bffc7Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (2 TMS) - 70eV, Positivesplash10-00dr-9310000000-a99f1bb4adbde62446d9Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, PositiveNot AvailableSpectrum
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (TMS_1_1) - 70eV, PositiveNot AvailableSpectrum
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (TBDMS_1_1) - 70eV, PositiveNot AvailableSpectrum
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (TBDMS_2_1) - 70eV, PositiveNot AvailableSpectrum
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 10V, Negative (Annotated)splash10-00di-9000000000-1578bea9a6b8b12e2f9bSpectrum
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 25V, Negative (Annotated)splash10-05fr-9000000000-0e443f81ee9c6c9b1fa5Spectrum
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 40V, Negative (Annotated)splash10-0ab9-9100000000-d23d6a0a35141ed9a950Spectrum
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 10V, Negativesplash10-01b9-6900000000-6d969044a89f00e1b46fSpectrum
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 20V, Negativesplash10-00di-9000000000-0f655174050263224e67Spectrum
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 30V, Negativesplash10-0ab9-9000000000-26a654fb133b28a816a0Spectrum
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 40V, Negativesplash10-0a4i-9000000000-7afd566e443d5fa38887Spectrum
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 50V, Negativesplash10-0a4i-9000000000-5c4795e954d5c3e9b74eSpectrum
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , negativesplash10-01b9-6900000000-6d969044a89f00e1b46fSpectrum
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , negativesplash10-00di-9000000000-0f655174050263224e67Spectrum
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , negativesplash10-0ab9-9000000000-26a654fb133b28a816a0Spectrum
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , negativesplash10-0a4i-9000000000-7afd566e443d5fa38887Spectrum
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , negativesplash10-0a4i-9000000000-5c4795e954d5c3e9b74eSpectrum
LC-MS/MSLC-MS/MS Spectrum - 40V, Negativesplash10-0a4i-9000000000-175b62a78de8d2295d7cSpectrum
LC-MS/MSLC-MS/MS Spectrum - 10V, Positivesplash10-00dm-9100000000-2118d1ea1507076c595cSpectrum
LC-MS/MSLC-MS/MS Spectrum - 20V, Positivesplash10-0007-9000000000-5ead13e9a754843f3de4Spectrum
LC-MS/MSLC-MS/MS Spectrum - 10V, Negativesplash10-00di-9000000000-04818c3d6cdaf0e910ebSpectrum
LC-MS/MSLC-MS/MS Spectrum - 40V, Positivesplash10-052f-9000000000-ea4a508808f00639e8c2Spectrum
LC-MS/MSLC-MS/MS Spectrum - 10V, Positivesplash10-0002-9100000000-e8e132c8d454aa8a0490Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-014i-5900000000-9b75ec910fa494f0ad6aSpectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-05di-9200000000-87a71ae13cf70d990bb1Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-004i-9000000000-e7ae04912b5ee745a992Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-014i-4900000000-0714f0bc5c29e5d61ac4Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-00di-9100000000-ecfebddbd3d8f7224994Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-05fr-9000000000-506ee5ec2100e13e09ceSpectrum
MSMass Spectrum (Electron Ionization)splash10-05di-9000000000-8d09b408489f7b1743b5Spectrum
1D NMR1H NMR SpectrumNot AvailableSpectrum
1D NMR13C NMR SpectrumNot AvailableSpectrum
1D NMR1H NMR SpectrumNot AvailableSpectrum
1D NMR13C NMR SpectrumNot AvailableSpectrum
1D NMR1H NMR SpectrumNot AvailableSpectrum
1D NMR13C NMR SpectrumNot AvailableSpectrum
1D NMR13C NMR SpectrumNot AvailableSpectrum
2D NMR[1H,13C] 2D NMR SpectrumNot AvailableSpectrum
Toxicity Profile
Route of ExposureNot Available
Mechanism of ToxicityMethylmalonic acid is a vital intermediate in the metabolism of fat and protein. Abnormalities in methylmalonic acid metabolism lead to methylmalonic aciduria. This metabolic disease is attributed to a block in the enzymatic conversion of methylmalonyl CoA to succinyl CoA. Accumulation of Methylmalonic acid in the body has been shown to be toxic.
MetabolismNot Available
Toxicity ValuesNot Available
Lethal DoseNot Available
Carcinogenicity (IARC Classification)No indication of carcinogenicity to humans (not listed by IARC).
Uses/SourcesThis is an endogenously produced metabolite found in the human body. It is used in metabolic reactions, catabolic reactions or waste generation.
Minimum Risk LevelNot Available
Health EffectsChronically high levels of methylmalonic acid are associated with at least 5 inborn errors of metabolism including: Malonyl CoA decarboxylase deficiency, Malonic Aciduria, Methylmalonate Semialdehyde Dehydrogenase Deficiency, Methylmalonic Aciduria and Methylmalonic Aciduria Due to Cobalamin-Related Disorders.
SymptomsNot Available
TreatmentNot Available
Concentrations
Not Available
DrugBank IDDB04183
HMDB IDHMDB0000202
FooDB IDFDB021905
Phenol Explorer IDNot Available
KNApSAcK IDNot Available
BiGG IDNot Available
BioCyc IDCPD-546
METLIN ID3712
PDB IDNot Available
Wikipedia LinkMethylmalonic_acid
Chemspider ID473
ChEBI ID30860
PubChem Compound ID487
Kegg Compound IDC02170
YMDB IDYMDB01567
ECMDB IDECMDB00202
References
Synthesis ReferenceNot Available
MSDSNot Available
General References
1. Edamura, Koji; Arai, Takeshi. Malonic and methylmalonic acids from b-hydroxypropionic acids. Jpn. Kokai Tokkyo Koho (1979), 5 pp.
2. van Asselt DZ, Karlietis MH, Poels PJ, de Jong JG, Wevers RA, Hoefnagels WH: Cerebrospinal fluid methylmalonic acid concentrations in neurological patients with low and normal serum cobalamin concentrations. Acta Neurol Scand. 1998 Jun;97(6):413-6.
3. Magera MJ, Helgeson JK, Matern D, Rinaldo P: Methylmalonic acid measured in plasma and urine by stable-isotope dilution and electrospray tandem mass spectrometry. Clin Chem. 2000 Nov;46(11):1804-10.
4. Nakamura E, Rosenberg LE, Tanaka K: Microdetermination of methylmalonic acid and other short chain dicarboxylic acids by gas chromatography: use in prenatal diagnosis of methylmalonic acidemia and in studies of isovaleric acidemia. Clin Chim Acta. 1976 Apr 15;68(2):127-40.
5. Jakobs C, Sweetman L, Nyhan WL: Hydroxy acid metabolites of branched-chain amino acids in amniotic fluid. Clin Chim Acta. 1984 Jul 16;140(2):157-66.
6. Coude MM, Charpentier C, Bonnefont JP, Cheron G, Kamoun P: Organic acids in aqueous humour and plasma: post mortem study in infants and diagnosis of enzymopathies. J Inherit Metab Dis. 1991;14(5):668-73.
7. 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.
8. Aleszczyk J: [Connection between changing the vitamin and immune status and the character of the throat microflora in patients with chronic tonsillitis]. Otolaryngol Pol. 2003;57(2):221-4.
9. Nagarajan S, Enns GM, Millan MT, Winter S, Sarwal MM: Management of methylmalonic acidaemia by combined liver-kidney transplantation. J Inherit Metab Dis. 2005;28(4):517-24.
10. Stabler SP, Marcell PD, Podell ER, Allen RH, Lindenbaum J: Assay of methylmalonic acid in the serum of patients with cobalamin deficiency using capillary gas chromatography-mass spectrometry. J Clin Invest. 1986 May;77(5):1606-12.
11. Jellum E, Stokke O, Eldjarn L: Combined use of gas chromatography, mass spectrometry, and computer in diagnosis and studies of metabolic disorders. Clin Chem. 1972 Aug;18(8):800-9.
12. Leupold D: [Methylmalonic aciduria. Classification, diagnosis and therapy (author's transl)]. Klin Wochenschr. 1977 Jan 15;55(2):57-63.
13. Vrethem M, Mattsson E, Hebelka H, Leerbeck K, Osterberg A, Landtblom AM, Balla B, Nilsson H, Hultgren M, Brattstrom L, Kagedal B: Increased plasma homocysteine levels without signs of vitamin B12 deficiency in patients with multiple sclerosis assessed by blood and cerebrospinal fluid homocysteine and methylmalonic acid. Mult Scler. 2003 Jun;9(3):239-45.
14. Bennett MJ, Ragni MC, Hood I, Hale DE: Comparison of post-mortem urinary and vitreous humour organic acids. Ann Clin Biochem. 1992 Sep;29 ( Pt 5):541-5.
15. Haurani FI, Hall CA, Rubin R: Megaloblastic anemia as a result of an abnormal transcobalamin II (Cardeza). J Clin Invest. 1979 Nov;64(5):1253-9.
16. Frenkel EP, Kitchens RL: Applicability of an enzymatic quantitation of methylmalonic, propionic, and acetic acids in normal and megaloblastic states. Blood. 1977 Jan;49(1):125-37.
17. 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.
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