Record Information
Version3.6
Creation Date2006-05-22 14:17:42 UTC
Update Date2013-05-29 19:34:51 UTC
HMDB IDHMDB02217
Secondary Accession NumbersNone
Metabolite Identification
Common Name(S)-Methylmalonic acid semialdehyde
DescriptionMethylmalonic semialdehyde is a metabolite in valine catabolism, inositol metabolism and propanoate metabolism. Methylmalonate-semialdehyde dehydrogenase (MMSDH) catalyses the NAD+ and coenzyme A-dependent conversion of methylmalonate semialdehyde to propionyl-CoA in the distal region of the L-valine catabolic pathway. MMSDH is located within the mitochondria; direct enzymatic assay of MMSDH is difficult since the substrate, methylmalonate semialdehyde, is both commercially unavailable and notoriously unstable as a b-keto acid. (PMID: 10947204 ).
Structure
Thumb
Synonyms
  1. (2S)-2-methyl-3-oxopropanoate
  2. (2S)-2-methyl-3-oxopropanoic acid
  3. (S)-Methylmalonate semialdehyde
Chemical FormulaC4H6O3
Average Molecular Weight102.0886
Monoisotopic Molecular Weight102.031694058
IUPAC Name(2S)-2-methyl-3-oxopropanoic acid
Traditional IUPAC Name(2S)-2-methyl-3-oxopropanoic acid
CAS Registry Number99043-16-0
SMILES
C[C@@H](C=O)C(O)=O
InChI Identifier
InChI=1S/C4H6O3/c1-3(2-5)4(6)7/h2-3H,1H3,(H,6,7)/t3-/m0/s1
InChI KeyVOKUMXABRRXHAR-VKHMYHEASA-N
Chemical Taxonomy
KingdomOrganic Compounds
Super ClassOrganic Acids and Derivatives
ClassCarboxylic Acids and Derivatives
Sub ClassCarboxylic Acid Derivatives
Other Descriptors
  • 2-methyl-3-oxopropanoic acid(ChEBI)
  • Aliphatic Acyclic Compounds
  • Oxo fatty acids(Lipidmaps)
  • semialdehyde(ChEBI)
Substituents
  • Aldehyde
Direct ParentCarboxylic Acids
Ontology
StatusExpected and Not Quantified
Origin
  • Endogenous
BiofunctionNot Available
ApplicationNot Available
Cellular locationsNot Available
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 solubility266 g/LALOGPS
logP0ALOGPS
logP0.053ChemAxon
logS0.42ALOGPS
pKa (strongest acidic)3.98ChemAxon
pKa (strongest basic)-7.2ChemAxon
physiological charge-1ChemAxon
hydrogen acceptor count3ChemAxon
hydrogen donor count1ChemAxon
polar surface area54.37ChemAxon
rotatable bond count2ChemAxon
refractivity22.64ChemAxon
polarizability9.16ChemAxon
Spectra
SpectraNot Available
Biological Properties
Cellular LocationsNot Available
Biofluid LocationsNot Available
Tissue LocationNot Available
Pathways
NameSMPDB LinkKEGG Link
Propanoate MetabolismSMP00016map00640
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 IDFDB022912
KNApSAcK IDNot Available
Chemspider ID4575365
KEGG Compound IDC06002
BioCyc IDNot Available
BiGG ID34698
Wikipedia LinkNot Available
NuGOwiki LinkHMDB02217
Metagene LinkHMDB02217
METLIN ID6553
PubChem Compound5462303
PDB IDNot Available
ChEBI ID27821
References
Synthesis ReferenceNot Available
Material Safety Data Sheet (MSDS)Not Available
General References
  1. Chambliss KL, Gray RG, Rylance G, Pollitt RJ, Gibson KM: Molecular characterization of methylmalonate semialdehyde dehydrogenase deficiency. J Inherit Metab Dis. 2000 Jul;23(5):497-504. Pubmed: 10947204
  2. Manning NJ, Pollitt RJ: Tracer studies of the interconversion of R- and S-methylmalonic semialdehydes in man. Biochem J. 1985 Oct 15;231(2):481-4. Pubmed: 4062908

Enzymes

Gene Name:
ALDH6A1
Uniprot ID:
Q02252
Reactions
(S)-Methylmalonic acid semialdehyde + Coenzyme A + NAD unknown Propionyl-CoA + Carbon dioxide + NADH + Hydrogen Iondetails
(S)-Methylmalonic acid semialdehyde + Coenzyme A + NAD unknown Methylmalonyl-CoA + NADH + Hydrogen Iondetails
Gene Name:
AOX1
Uniprot ID:
Q06278
Reactions
Methylmalonic acid + Hydrogen peroxide unknown (S)-Methylmalonic acid semialdehyde + Oxygen + Waterdetails
Gene Name:
ABAT
Uniprot ID:
P80404
Reactions
(S)-b-aminoisobutyric acid + Oxoglutaric acid unknown (S)-Methylmalonic acid semialdehyde + L-Glutamic aciddetails
Gene Name:
ALDH9A1
Uniprot ID:
P49189
Reactions
(S)-Methylmalonic acid semialdehyde + NAD + Water unknown Methylmalonic acid + NADH + Hydrogen Iondetails
Gene Name:
ALDH7A1
Uniprot ID:
P49419
Reactions
(S)-Methylmalonic acid semialdehyde + NAD + Water unknown Methylmalonic acid + NADH + Hydrogen Iondetails
Gene Name:
ALDH2
Uniprot ID:
P05091
Reactions
(S)-Methylmalonic acid semialdehyde + NAD + Water unknown Methylmalonic acid + NADH + Hydrogen Iondetails
Gene Name:
ALDH3A2
Uniprot ID:
P51648
Reactions
(S)-Methylmalonic acid semialdehyde + NAD + Water unknown Methylmalonic acid + NADH + Hydrogen Iondetails
Gene Name:
ALDH1B1
Uniprot ID:
P30837
Reactions
(S)-Methylmalonic acid semialdehyde + NAD + Water unknown Methylmalonic acid + NADH + Hydrogen Iondetails
Gene Name:
HSD17B10
Uniprot ID:
Q99714
Gene Name:
EHHADH
Uniprot ID:
Q08426
Reactions
(S)-3-Hydroxyisobutyric acid + NAD unknown (S)-Methylmalonic acid semialdehyde + NADH + Hydrogen Iondetails
Gene Name:
HADH
Uniprot ID:
Q16836
Reactions
(S)-3-Hydroxyisobutyric acid + NAD unknown (S)-Methylmalonic acid semialdehyde + NADH + Hydrogen Iondetails
Gene Name:
HIBADH
Uniprot ID:
P31937
Reactions
(S)-3-Hydroxyisobutyric acid + NAD unknown (S)-Methylmalonic acid semialdehyde + NADH + Hydrogen Iondetails
Gene Name:
HSD17B4
Uniprot ID:
P51659