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Record Information
Version3.6
Creation Date2005-11-16 15:48:42 UTC
Update Date2016-02-11 01:04:08 UTC
HMDB IDHMDB01269
Secondary Accession NumbersNone
Metabolite Identification
Common NameMethylmalonyl-CoA
DescriptionMethylmalonyl-CoA is an intermediate in the metabolism of Propanoate. It is a substrate for Malonyl-CoA decarboxylase (mitochondrial), Methylmalonyl-CoA mutase (mitochondrial) and Methylmalonyl-CoA epimerase (mitochondrial).
Structure
Thumb
Synonyms
ValueSource
(R)-Methylmalonyl-CoAHMDB
(R)-Methylmalonyl-coenzyme AHMDB
(S)-2-Methyl-3-oxopropanoyl-CoAHMDB
(S)-Methyl-malonyl-CoAHMDB
(S)-Methyl-malonyl-coenzyme AHMDB
CH3-Malonyl-CoAHMDB
CH3-Malonyl-coenzyme AHMDB
D-Methylmalonyl-CoAHMDB
D-Methylmalonyl-coenzyme AHMDB
L-Methylmalonyl-CoAHMDB
L-Methylmalonyl-coenzyme AHMDB
Methyl-malonyl-coenzyme AHMDB
Chemical FormulaC25H40N7O19P3S
Average Molecular Weight867.607
Monoisotopic Molecular Weight867.131252359
IUPAC Name(2S)-3-{[2-(3-{3-[({[({[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-4-hydroxy-3-(phosphonooxy)oxolan-2-yl]methoxy}(hydroxy)phosphoryl)oxy](hydroxy)phosphoryl}oxy)methyl]-2-hydroxy-3-methylbutanamido}propanamido)ethyl]sulfanyl}-2-methyl-3-oxopropanoic acid
Traditional Name(2S)-3-({2-[3-(3-{[({[(2R,3S,4R,5R)-5-(6-aminopurin-9-yl)-4-hydroxy-3-(phosphonooxy)oxolan-2-yl]methoxy(hydroxy)phosphoryl}oxy(hydroxy)phosphoryl)oxy]methyl}-2-hydroxy-3-methylbutanamido)propanamido]ethyl}sulfanyl)-2-methyl-3-oxopropanoic acid
CAS Registry Number104809-02-1
SMILES
C[C@@H](C(O)=O)C(=O)SCCNC(=O)CCNC(=O)C(O)C(C)(C)COP(O)(=O)OP(O)(=O)OC[C@H]1O[C@H]([C@H](O)[C@@H]1OP(O)(O)=O)N1C=NC2=C1N=CN=C2N
InChI Identifier
InChI=1S/C25H40N7O19P3S/c1-12(23(37)38)24(39)55-7-6-27-14(33)4-5-28-21(36)18(35)25(2,3)9-48-54(45,46)51-53(43,44)47-8-13-17(50-52(40,41)42)16(34)22(49-13)32-11-31-15-19(26)29-10-30-20(15)32/h10-13,16-18,22,34-35H,4-9H2,1-3H3,(H,27,33)(H,28,36)(H,37,38)(H,43,44)(H,45,46)(H2,26,29,30)(H2,40,41,42)/t12-,13+,16+,17+,18?,22+/m0/s1
InChI KeyInChIKey=MZFOKIKEPGUZEN-PNEPRAIJSA-N
Chemical Taxonomy
DescriptionThis compound belongs to the class of organic compounds known as acyl coas. These are organic compounds containing a coenzyme A substructure linked to an acyl chain.
KingdomOrganic compounds
Super ClassLipids and lipid-like molecules
ClassFatty Acyls
Sub ClassFatty acyl thioesters
Direct ParentAcyl CoAs
Alternative Parents
Substituents
  • Coenzyme a or derivatives
  • Purine ribonucleoside diphosphate
  • Purine ribonucleoside 3',5'-bisphosphate
  • N-glycosyl compound
  • Glycosyl compound
  • Beta amino acid or derivatives
  • Organic pyrophosphate
  • Monosaccharide phosphate
  • 6-aminopurine
  • Purine
  • Imidazopyrimidine
  • Monoalkyl phosphate
  • Aminopyrimidine
  • Imidolactam
  • Alkyl phosphate
  • 1,3-dicarbonyl compound
  • Pyrimidine
  • Primary aromatic amine
  • Phosphoric acid ester
  • Organic phosphoric acid derivative
  • Organic phosphate
  • N-substituted imidazole
  • N-acyl-amine
  • Monosaccharide
  • Fatty amide
  • Saccharide
  • Heteroaromatic compound
  • Oxolane
  • Imidazole
  • Azole
  • Thiocarboxylic acid ester
  • Secondary carboxylic acid amide
  • Secondary alcohol
  • Carboxamide group
  • Oxacycle
  • Azacycle
  • Organoheterocyclic compound
  • Sulfenyl compound
  • Thioether
  • Thiocarboxylic acid or derivatives
  • Monocarboxylic acid or derivatives
  • Carboxylic acid
  • Carboxylic acid derivative
  • Carboxylic acid amide
  • Hydrocarbon derivative
  • Primary amine
  • Organosulfur compound
  • Organooxygen compound
  • Organonitrogen compound
  • Carbonyl group
  • Amine
  • Alcohol
  • Aromatic heteropolycyclic compound
Molecular FrameworkAromatic heteropolycyclic compounds
External DescriptorsNot Available
Ontology
StatusExpected but not Quantified
Origin
  • Endogenous
  • Food
Biofunction
  • Cell signaling
  • Component of Propanoate metabolism
  • Fuel and energy storage
  • Fuel or energy source
  • Membrane integrity/stability
Application
  • Nutrients
  • Stabilizers
  • Surfactants and Emulsifiers
Cellular locations
  • Extracellular
  • Membrane
  • Mitochondria
Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting PointNot AvailableNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Predicted Properties
PropertyValueSource
Water Solubility3.57 mg/mLALOGPS
logP-0.6ALOGPS
logP-6.5ChemAxon
logS-2.4ALOGPS
pKa (Strongest Acidic)0.82ChemAxon
pKa (Strongest Basic)4.97ChemAxon
Physiological Charge-5ChemAxon
Hydrogen Acceptor Count19ChemAxon
Hydrogen Donor Count10ChemAxon
Polar Surface Area400.93 Å2ChemAxon
Rotatable Bond Count22ChemAxon
Refractivity183.13 m3·mol-1ChemAxon
Polarizability75.35 Å3ChemAxon
Number of Rings3ChemAxon
Bioavailability0ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectra
Spectra
Spectrum TypeDescriptionSplash Key
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-000i-4911000020-3f5d871d28d1f2657e02View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-000i-1913000000-eb05e99fd73a630e7c4aView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-000i-3911000000-ec1f0018f511db865738View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-017j-9811140550-4b48b6e3d5690659e256View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-003r-4911110010-d9e5414e595f184f73caView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-057i-6900100000-464114c146993e32adb3View in MoNA
Biological Properties
Cellular Locations
  • Extracellular
  • Membrane
  • Mitochondria
Biofluid LocationsNot Available
Tissue LocationNot Available
Pathways
NameSMPDB LinkKEGG Link
Valine, Leucine and Isoleucine DegradationSMP00032map00280
Normal Concentrations
Not Available
Abnormal Concentrations
Not Available
Associated Disorders and Diseases
Disease ReferencesNone
Associated OMIM IDsNone
DrugBank IDNot Available
DrugBank Metabolite IDNot Available
Phenol Explorer Compound IDNot Available
Phenol Explorer Metabolite IDNot Available
FoodDB IDFDB022523
KNApSAcK IDNot Available
Chemspider ID388424
KEGG Compound IDC00683
BioCyc IDMETHYL-MALONYL-COA
BiGG ID37090
Wikipedia LinkMethylmalonyl-CoA
NuGOwiki LinkHMDB01269
Metagene LinkHMDB01269
METLIN ID462
PubChem Compound439291
PDB IDNot Available
ChEBI ID15466
References
Synthesis ReferenceNot Available
Material Safety Data Sheet (MSDS)Download (PDF)
General References
  1. Watkins D, Rosenblatt DS: Inborn errors of cobalamin absorption and metabolism. Am J Med Genet C Semin Med Genet. 2011 Feb 15;157C(1):33-44. doi: 10.1002/ajmg.c.30288. Epub 2011 Feb 10. [21312325 ]
  2. Quadros EV: Advances in the understanding of cobalamin assimilation and metabolism. Br J Haematol. 2010 Jan;148(2):195-204. doi: 10.1111/j.1365-2141.2009.07937.x. Epub 2009 Oct 12. [19832808 ]
  3. Rincon A, Aguado C, Desviat LR, Sanchez-Alcudia R, Ugarte M, Perez B: Propionic and methylmalonic acidemia: antisense therapeutics for intronic variations causing aberrantly spliced messenger RNA. Am J Hum Genet. 2007 Dec;81(6):1262-70. [17966092 ]
  4. Morrow G 3rd, Barness LA, Cardinale GJ, Abeles RH, Flaks JG: Congenital methylmalonic acidemia: enzymatic evidence for two forms of the disease. Proc Natl Acad Sci U S A. 1969 May;63(1):191-7. [5257962 ]
  5. Loewy AD, Niles KM, Anastasio N, Watkins D, Lavoie J, Lerner-Ellis JP, Pastinen T, Trasler JM, Rosenblatt DS: Epigenetic modification of the gene for the vitamin B(12) chaperone MMACHC can result in increased tumorigenicity and methionine dependence. Mol Genet Metab. 2009 Apr;96(4):261-7. doi: 10.1016/j.ymgme.2008.12.011. Epub 2009 Feb 5. [19200761 ]
  6. Ludwig ML, Matthews RG: Structure-based perspectives on B12-dependent enzymes. Annu Rev Biochem. 1997;66:269-313. [9242908 ]
  7. Shevell MI, Matiaszuk N, Ledley FD, Rosenblatt DS: Varying neurological phenotypes among muto and mut- patients with methylmalonylCoA mutase deficiency. Am J Med Genet. 1993 Mar 1;45(5):619-24. [7681251 ]
  8. Gu P, Welch WH, Guo L, Schegg KM, Blomquist GJ: Characterization of a novel microsomal fatty acid synthetase (FAS) compared to a cytosolic FAS in the housefly, Musca domestica. Comp Biochem Physiol B Biochem Mol Biol. 1997 Oct;118(2):447-56. [9440236 ]

Enzymes

General function:
Involved in oxidoreductase activity
Specific function:
Plays a role in valine and pyrimidine metabolism. Binds fatty acyl-CoA.
Gene Name:
ALDH6A1
Uniprot ID:
Q02252
Molecular weight:
57839.31
Reactions
(S)-Methylmalonic acid semialdehyde + Coenzyme A + NAD → Methylmalonyl-CoA + NADH + Hydrogen Iondetails
General function:
Amino acid transport and metabolism
Specific function:
Not Available
Gene Name:
MCEE
Uniprot ID:
Q96PE7
Molecular weight:
18748.755
Reactions
Methylmalonyl-CoA → S-Methylmalonyl-CoAdetails
General function:
Involved in isomerase activity
Specific function:
Involved in the degradation of several amino acids, odd-chain fatty acids and cholesterol via propionyl-CoA to the tricarboxylic acid cycle. MCM has different functions in other species.
Gene Name:
MUT
Uniprot ID:
P22033
Molecular weight:
83133.755
Reactions
Methylmalonyl-CoA → Succinyl-CoAdetails
General function:
Involved in ligase activity
Specific function:
Not Available
Gene Name:
PCCB
Uniprot ID:
P05166
Molecular weight:
58215.13
General function:
Involved in catalytic activity
Specific function:
Not Available
Gene Name:
PCCA
Uniprot ID:
P05165
Molecular weight:
80058.295