Human Metabolome Database Version 3.5

Showing metabocard for (S)-Methylmalonic acid semialdehyde (HMDB02217)

Record Information
Version 3.5
Creation Date 2006-05-22 08:17:42 -0600
Update Date 2013-05-29 13:34:51 -0600
HMDB ID HMDB02217
Secondary Accession Numbers None
Metabolite Identification
Common Name (S)-Methylmalonic acid semialdehyde
Description Methylmalonic 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 Link_out).
Structure Thumb
Download: MOL | SDF | PDB | SMILES | InChI
Display: 2D Structure | 3D Structure
Synonyms
  1. (2S)-2-methyl-3-oxopropanoate
  2. (2S)-2-methyl-3-oxopropanoic acid
  3. (S)-Methylmalonate semialdehyde
Chemical Formula C4H6O3
Average Molecular Weight 102.0886
Monoisotopic Molecular Weight 102.031694058
IUPAC Name (2S)-2-methyl-3-oxopropanoic acid
Traditional IUPAC Name (2S)-2-methyl-3-oxopropanoic acid
CAS Registry Number 99043-16-0
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 Key VOKUMXABRRXHAR-VKHMYHEASA-N
Chemical Taxonomy
Kingdom Organic Compounds
Super Class Organic Acids and Derivatives
Class Carboxylic Acids and Derivatives
Sub Class Carboxylic Acid Derivatives
Other Descriptors
  • 2-methyl-3-oxopropanoic acid(ChEBI)
  • Aliphatic Acyclic Compounds
  • Oxo fatty acids(Lipidmaps)
  • semialdehyde(ChEBI)
Substituents
  • Aldehyde
Direct Parent Carboxylic Acids
Ontology
Status Expected and Not Quantified
Origin
  • Endogenous
Biofunction Not Available
Application Not Available
Cellular locations Not Available
Physical Properties
State Solid
Experimental Properties
Property Value Reference
Melting Point Not Available Not Available
Boiling Point Not Available Not Available
Water Solubility Not Available Not Available
LogP Not Available Not Available
Predicted Properties
Property Value Source
Water Solubility 266 g/L ALOGPS
LogP -0.00 ALOGPS
LogP 0.053 ChemAxon
LogS 0.42 ALOGPS
pKa (strongest acidic) 3.98 ChemAxon
pKa (strongest basic) -7.2 ChemAxon
Hydrogen Acceptor Count 3 ChemAxon
Hydrogen Donor Count 1 ChemAxon
Polar Surface Area 54.37 A2 ChemAxon
Rotatable Bond Count 2 ChemAxon
Refractivity 22.64 ChemAxon
Polarizability 9.16 ChemAxon
Formal Charge 0 ChemAxon
Physiological Charge -1 ChemAxon
Spectra
Not Available
Biological Properties
Cellular Locations Not Available
Biofluid Locations Not Available
Tissue Location Not Available
Pathways
Name SMPDB Link KEGG Link
Valine, Leucine and Isoleucine Degradation SMP00032 map00280 Link_out
Propanoate Metabolism SMP00016 map00640 Link_out
Normal Concentrations
Not Available
Abnormal Concentrations
Not Available
Associated Disorders and Diseases
Disease References None
Associated OMIM IDs None
DrugBank ID Not Available
DrugBank Metabolite ID Not Available
Phenol Explorer Compound ID Not Available
Phenol Explorer Metabolite ID Not Available
FoodDB ID FDB022912
KNApSAcK ID Not Available
Chemspider ID 4575365 Link_out
KEGG Compound ID C06002 Link_out
BioCyc ID Not Available
BiGG ID 34698 Link_out
Wikipedia Link Not Available
NuGOwiki Link HMDB02217 Link_out
Metagene Link HMDB02217 Link_out
METLIN ID 6553 Link_out
PubChem Compound 5462303 Link_out
PDB ID Not Available
ChEBI ID 27821 Link_out
References
Synthesis Reference Not Available
Material Safety Data Sheet (MSDS) Not Available
General References
  1. 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 Link_out
  2. 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 Link_out

Enzymes
Name: Methylmalonate-semialdehyde dehydrogenase [acylating], mitochondrial
Reactions:
(S)-Methylmalonic acid semialdehyde + Coenzyme A + NAD unknown Propionyl-CoA + Carbon dioxide + NADH + Hydrogen Ion details
(S)-Methylmalonic acid semialdehyde + Coenzyme A + NAD unknown Methylmalonyl-CoA + NADH + Hydrogen Ion details
Gene Name: ALDH6A1
Uniprot ID: Q02252 Link_out
Protein Sequence: FASTA
Gene Sequence: FASTA
Name: Aldehyde oxidase
Reactions:
Methylmalonic acid + Hydrogen peroxide unknown (S)-Methylmalonic acid semialdehyde + Oxygen + Water details
Gene Name: AOX1
Uniprot ID: Q06278 Link_out
Protein Sequence: FASTA
Gene Sequence: FASTA
Name: 4-aminobutyrate aminotransferase, mitochondrial
Reactions:
(S)-b-aminoisobutyric acid + Oxoglutaric acid unknown (S)-Methylmalonic acid semialdehyde + L-Glutamic acid details
Gene Name: ABAT
Uniprot ID: P80404 Link_out
Protein Sequence: FASTA
Gene Sequence: FASTA
Name: 4-trimethylaminobutyraldehyde dehydrogenase
Reactions:
(S)-Methylmalonic acid semialdehyde + NAD + Water unknown Methylmalonic acid + NADH + Hydrogen Ion details
Gene Name: ALDH9A1
Uniprot ID: P49189 Link_out
Protein Sequence: FASTA
Gene Sequence: FASTA
Name: Alpha-aminoadipic semialdehyde dehydrogenase
Reactions:
(S)-Methylmalonic acid semialdehyde + NAD + Water unknown Methylmalonic acid + NADH + Hydrogen Ion details
Gene Name: ALDH7A1
Uniprot ID: P49419 Link_out
Protein Sequence: FASTA
Gene Sequence: FASTA
Name: Aldehyde dehydrogenase family 1 member A3
Reactions: Not Available
Gene Name: ALDH1A3
Uniprot ID: P47895 Link_out
Protein Sequence: FASTA
Gene Sequence: FASTA
Name: Aldehyde dehydrogenase, mitochondrial
Reactions:
(S)-Methylmalonic acid semialdehyde + NAD + Water unknown Methylmalonic acid + NADH + Hydrogen Ion details
Gene Name: ALDH2
Uniprot ID: P05091 Link_out
Protein Sequence: FASTA
Gene Sequence: FASTA
Name: Fatty aldehyde dehydrogenase
Reactions:
(S)-Methylmalonic acid semialdehyde + NAD + Water unknown Methylmalonic acid + NADH + Hydrogen Ion details
Gene Name: ALDH3A2
Uniprot ID: P51648 Link_out
Protein Sequence: FASTA
Gene Sequence: FASTA
Name: Aldehyde dehydrogenase X, mitochondrial
Reactions:
(S)-Methylmalonic acid semialdehyde + NAD + Water unknown Methylmalonic acid + NADH + Hydrogen Ion details
Gene Name: ALDH1B1
Uniprot ID: P30837 Link_out
Protein Sequence: FASTA
Gene Sequence: FASTA
Name: 3-hydroxyacyl-CoA dehydrogenase type-2
Reactions: Not Available
Gene Name: HSD17B10
Uniprot ID: Q99714 Link_out
Protein Sequence: FASTA
Gene Sequence: FASTA
Name: Peroxisomal bifunctional enzyme
Reactions:
(S)-3-Hydroxyisobutyric acid + NAD unknown (S)-Methylmalonic acid semialdehyde + NADH + Hydrogen Ion details
Gene Name: EHHADH
Uniprot ID: Q08426 Link_out
Protein Sequence: FASTA
Gene Sequence: FASTA
Name: Hydroxyacyl-coenzyme A dehydrogenase, mitochondrial
Reactions:
(S)-3-Hydroxyisobutyric acid + NAD unknown (S)-Methylmalonic acid semialdehyde + NADH + Hydrogen Ion details
Gene Name: HADH
Uniprot ID: Q16836 Link_out
Protein Sequence: FASTA
Gene Sequence: FASTA
Name: 3-hydroxyisobutyrate dehydrogenase, mitochondrial
Reactions:
(S)-3-Hydroxyisobutyric acid + NAD unknown (S)-Methylmalonic acid semialdehyde + NADH + Hydrogen Ion details
Gene Name: HIBADH
Uniprot ID: P31937 Link_out
Protein Sequence: FASTA
Gene Sequence: FASTA
Name: Peroxisomal multifunctional enzyme type 2
Reactions: Not Available
Gene Name: HSD17B4
Uniprot ID: P51659 Link_out
Protein Sequence: FASTA
Gene Sequence: FASTA