Showing metabocard for Methylmalonic acid (BMDB0000202)

IdentificationTaxonomyPhysical propertiesPathwaysSpectraConcentrationsLinksReferencesenzymes (2) Show 2 proteinsXML
Show more...Show more...
Record Information
Version1.0
Creation Date2016-09-30 22:26:39 UTC
Update Date2020-06-04 19:31:06 UTC
BMDB IDBMDB0000202
Secondary Accession Numbers
  • BMDB00202
Metabolite Identification
Common NameMethylmalonic acid
DescriptionMethylmalonic acid, also known as 2-methylmalonate or methyl-propanedioate, belongs to the class of organic compounds known as dicarboxylic acids and derivatives. These are organic compounds containing exactly two carboxylic acid groups. Methylmalonic acid exists as a solid, very hydrophobic, practically insoluble (in water), and relatively neutral molecule. Methylmalonic acid exists in all living species, ranging from bacteria to humans. In cattle, methylmalonic acid is involved in the metabolic pathway called the vitamin K metabolism pathway. Methylmalonic acid is a potentially toxic compound. Methylmalonic acid has been found to be associated with several diseases known as eosinophilic esophagitis, combined malonic and methylmalonic aciduria, alzheimer's disease, and methylmalonic aciduria mitochondrial encephelopathy leigh-like; also methylmalonic acid has been linked to the inborn metabolic disorders including vitamin B12 deficiency.
Structure
Thumb
MOLSDFPDBSMILESInChI
Synonyms
Chemical FormulaC4H6O4
Average Molecular Weight118.088
Monoisotopic Molecular Weight118.02660868
IUPAC Name2-methylpropanedioic acid
Traditional Namemethylmalonic acid
CAS Registry Number516-05-2
SMILES
CC(C(O)=O)C(O)=O
InChI Identifier
InChI=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
Ontology
StatusDetected and Quantified
Origin
  • Endogenous
BiofunctionNot Available
ApplicationNot Available
Cellular locations
  • Cytoplasm
Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting Point135 °CNot Available
Boiling PointNot AvailableNot Available
Water Solubility679.0 mg/mLNot Available
LogPNot AvailableNot Available
Predicted Properties
PropertyValueSource
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
BioavailabilityYesChemAxon
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) (2 TMS)splash10-0002-1910000000-cf95dd8a481761a28664View in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (2 TMS)splash10-006t-8900000000-4d8795e7e4300cc7ceffView in MoNA
GC-MSGC-MS Spectrum - GC-MS (2 TMS)splash10-00lr-5940000000-56b86ada220d335ce30aView in MoNA
GC-MSGC-MS Spectrum - GC-MS (3 TMS)splash10-001i-9301000000-54e5247d046de566ac1eView in MoNA
GC-MSGC-MS Spectrum - EI-B (Non-derivatized)splash10-004i-9000000000-b9ee59dddf90dfc7c461View in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-0002-1910000000-cf95dd8a481761a28664View in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-006t-8900000000-4d8795e7e4300cc7ceffView in MoNA
GC-MSGC-MS Spectrum - GC-MS (Non-derivatized)splash10-001i-9301000000-54e5247d046de566ac1eView in MoNA
GC-MSGC-MS Spectrum - GC-MS (Non-derivatized)splash10-00lr-5940000000-56b86ada220d335ce30aView in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-0002-0900000000-684a11ce99cccbd9194aView in MoNA
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positivesplash10-0g4i-9400000000-baa4ea6d613ebb8bffc7View in MoNA
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (2 TMS) - 70eV, Positivesplash10-00dr-9310000000-a99f1bb4adbde62446d9View in MoNA
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, PositiveNot AvailableView in JSpectraViewer
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (TMS_1_1) - 70eV, PositiveNot AvailableView in JSpectraViewer
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (TBDMS_1_1) - 70eV, PositiveNot AvailableView in JSpectraViewer
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (TBDMS_2_1) - 70eV, PositiveNot AvailableView in JSpectraViewer
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 10V, Negative (Annotated)splash10-00di-9000000000-1578bea9a6b8b12e2f9bView in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 25V, Negative (Annotated)splash10-05fr-9000000000-0e443f81ee9c6c9b1fa5View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 40V, Negative (Annotated)splash10-0ab9-9100000000-d23d6a0a35141ed9a950View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 10V, Negativesplash10-01b9-6900000000-6d969044a89f00e1b46fView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 20V, Negativesplash10-00di-9000000000-0f655174050263224e67View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 30V, Negativesplash10-0ab9-9000000000-26a654fb133b28a816a0View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 40V, Negativesplash10-0a4i-9000000000-7afd566e443d5fa38887View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 50V, Negativesplash10-0a4i-9000000000-5c4795e954d5c3e9b74eView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , negativesplash10-01b9-6900000000-6d969044a89f00e1b46fView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , negativesplash10-00di-9000000000-0f655174050263224e67View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , negativesplash10-0ab9-9000000000-26a654fb133b28a816a0View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , negativesplash10-0a4i-9000000000-7afd566e443d5fa38887View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , negativesplash10-0a4i-9000000000-5c4795e954d5c3e9b74eView in MoNA
LC-MS/MSLC-MS/MS Spectrum - 40V, Negativesplash10-0a4i-9000000000-175b62a78de8d2295d7cView in MoNA
LC-MS/MSLC-MS/MS Spectrum - 10V, Positivesplash10-00dm-9100000000-2118d1ea1507076c595cView in MoNA
LC-MS/MSLC-MS/MS Spectrum - 20V, Positivesplash10-0007-9000000000-5ead13e9a754843f3de4View in MoNA
LC-MS/MSLC-MS/MS Spectrum - 10V, Negativesplash10-00di-9000000000-04818c3d6cdaf0e910ebView in MoNA
LC-MS/MSLC-MS/MS Spectrum - 40V, Positivesplash10-052f-9000000000-ea4a508808f00639e8c2View in MoNA
LC-MS/MSLC-MS/MS Spectrum - 10V, Positivesplash10-0002-9100000000-e8e132c8d454aa8a0490View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-014i-5900000000-9b75ec910fa494f0ad6aView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-05di-9200000000-87a71ae13cf70d990bb1View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-004i-9000000000-e7ae04912b5ee745a992View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-014i-4900000000-0714f0bc5c29e5d61ac4View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-00di-9100000000-ecfebddbd3d8f7224994View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-05fr-9000000000-506ee5ec2100e13e09ceView in MoNA
MSMass Spectrum (Electron Ionization)splash10-05di-9000000000-8d09b408489f7b1743b5View in MoNA
1D NMR1H NMR Spectrum (1D, 500 MHz, H2O, experimental)Not AvailableView in JSpectraViewer
1D NMR13C NMR Spectrum (1D, 125 MHz, H2O, experimental)Not AvailableView in JSpectraViewer
1D NMR1H NMR Spectrum (1D, 400 MHz, DMSO-d6, experimental)Not AvailableView in JSpectraViewer
1D NMR13C NMR Spectrum (1D, 22.53 MHz, DMSO-d6, experimental)Not AvailableView in JSpectraViewer
1D NMR1H NMR Spectrum (1D, D2O, experimental)Not AvailableView in JSpectraViewer
1D NMR13C NMR Spectrum (1D, D2O, experimental)Not AvailableView in JSpectraViewer
1D NMR13C NMR Spectrum (1D, 400 MHz, H2O, experimental)Not AvailableView in JSpectraViewer
2D NMR[1H, 13C]-HSQC NMR Spectrum (2D, 600 MHz, H2O, experimental)Not AvailableView in JSpectraViewer
Biological Properties
Cellular Locations
  • Cytoplasm
Biospecimen Locations
  • Blood
  • Kidney
  • Liver
  • Mammary Gland
  • Milk
  • Placenta
  • Ruminal Fluid
Pathways
Normal Concentrations
Abnormal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected but not QuantifiedNot QuantifiedNot SpecifiedNot Specified
Sub/clinical ketosis		 details
HMDB IDHMDB0000202
DrugBank IDDB04183
Phenol Explorer Compound IDNot Available
FooDB IDFDB021905
KNApSAcK IDNot Available
Chemspider ID473
KEGG Compound IDC02170
BioCyc IDCPD-546
BiGG IDNot Available
Wikipedia LinkMethylmalonic_acid
METLIN ID3712
PubChem Compound487
PDB IDNot Available
ChEBI ID30860
References
Synthesis ReferenceEdamura, Koji; Arai, Takeshi. Malonic and methylmalonic acids from b-hydroxypropionic acids. Jpn. Kokai Tokkyo Koho (1979), 5 pp.
Material Safety Data Sheet (MSDS)Not Available
General References
  1. Sun HZ, Shi K, Wu XH, Xue MY, Wei ZH, Liu JX, Liu HY: Lactation-related metabolic mechanism investigated based on mammary gland metabolomics and 4 biofluids' metabolomics relationships in dairy cows. BMC Genomics. 2017 Dec 2;18(1):936. doi: 10.1186/s12864-017-4314-1. [PubMed:29197344 ]
  2. Antunes-Fernandes EC, van Gastelen S, Dijkstra J, Hettinga KA, Vervoort J: Milk metabolome relates enteric methane emission to milk synthesis and energy metabolism pathways. J Dairy Sci. 2016 Aug;99(8):6251-6262. doi: 10.3168/jds.2015-10248. Epub 2016 May 26. [PubMed:27236769 ]

Enzymes

Enzyme Details
1. Aldehyde oxidase 1
General function:
Nucleotide transport and metabolism
Specific function:
Oxidase with broad substrate specificity, oxidizing aromatic azaheterocycles, such as N1-methylnicotinamide, N-methylphthalazinium and phthalazine, as well as aldehydes, such as benzaldehyde, retinal, pyridoxal, and vanillin. Plays a key role in the metabolism of xenobiotics and drugs containing aromatic azaheterocyclic substituents. Is probably involved in the regulation of reactive oxygen species homeostasis. May be a prominent source of superoxide generation via the one-electron reduction of molecular oxygen. Also may catalyze nitric oxide (NO) production via the reduction of nitrite to NO with NADH or aldehyde as electron donor. May play a role in adipogenesis.
Gene Name:
AOX1
Uniprot ID:
P48034
Molecular weight:
147611.0
Reactions
(S)-Methylmalonic acid semialdehyde + Water + Oxygen → Methylmalonic acid + Hydrogen peroxidedetails
Enzyme Details
2. Aldehyde dehydrogenase, mitochondrial
General function:
Energy production and conversion
Specific function:
Not Available
Gene Name:
ALDH2
Uniprot ID:
P20000
Molecular weight:
56653.0
Reactions
(S)-Methylmalonic acid semialdehyde + NAD + Water → Methylmalonic acid + NADHdetails