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Record Information
Version5.0
StatusExpected but not Quantified
Creation Date2007-05-23 15:44:17 UTC
Update Date2023-02-21 17:17:20 UTC
HMDB IDHMDB0006556
Secondary Accession Numbers
  • HMDB06556
Metabolite Identification
Common NameL-4-Hydroxyglutamate semialdehyde
DescriptionL-4-Hydroxyglutamate semialdehyde, also known as (4R)-4-hydroxy-5-oxo-L-norvaline, belongs to the class of organic compounds known as l-alpha-amino acids. These are alpha amino acids which have the L-configuration of the alpha-carbon atom. L-4-Hydroxyglutamate semialdehyde is a very strong basic compound (based on its pKa). A glutamic semialdehyde that is L-glutamic 5-semialdehyde substituted by a hydroxy group at position 4. L-4-Hydroxyglutamate semialdehyde exists in all living species, ranging from bacteria to humans. Within humans, L-4-hydroxyglutamate semialdehyde participates in a number of enzymatic reactions. In particular, L-4-hydroxyglutamate semialdehyde can be biosynthesized from 4-hydroxy-L-glutamic acid through the action of the enzyme proline dehydrogenase 1, mitochondrial. In addition, L-4-hydroxyglutamate semialdehyde can be biosynthesized from 4-hydroxy-L-glutamic acid through its interaction with the enzyme Delta-1-pyrroline-5-carboxylate dehydrogenase, mitochondrial. In humans, L-4-hydroxyglutamate semialdehyde is involved in the metabolic disorder called hyperornithinemia with gyrate atrophy (hoga).
Structure
Thumb
Synonyms
ValueSource
(4R)-4-Hydroxy-5-oxo-L-norvalineChEBI
4-Amino-3,4-dideoxy-D-erythro-penturonic acidChEBI
4-Amino-3,4-dideoxy-D-erythro-penturonateGenerator
L-4-Hydroxyglutamic acid semialdehydeGenerator
Chemical FormulaC5H9NO4
Average Molecular Weight147.1293
Monoisotopic Molecular Weight147.053157781
IUPAC Name(2S,4R)-2-amino-4-hydroxy-5-oxopentanoic acid
Traditional Name(2S,4R)-2-amino-4-hydroxy-5-oxopentanoic acid
CAS Registry NumberNot Available
SMILES
N[C@@H](C[C@@H](O)C=O)C(O)=O
InChI Identifier
InChI=1S/C5H9NO4/c6-4(5(9)10)1-3(8)2-7/h2-4,8H,1,6H2,(H,9,10)/t3-,4+/m1/s1
InChI KeyXCXUZPXOFFRGGP-DMTCNVIQSA-N
Chemical Taxonomy
Description Belongs to the class of organic compounds known as l-alpha-amino acids. These are alpha amino acids which have the L-configuration of the alpha-carbon atom.
KingdomOrganic compounds
Super ClassOrganic acids and derivatives
ClassCarboxylic acids and derivatives
Sub ClassAmino acids, peptides, and analogues
Direct ParentL-alpha-amino acids
Alternative Parents
Substituents
  • L-alpha-amino acid
  • Short-chain hydroxy acid
  • Fatty acid
  • 1,3-aminoalcohol
  • Alpha-hydroxyaldehyde
  • Secondary alcohol
  • Amino acid
  • Carboxylic acid
  • Monocarboxylic acid or derivatives
  • Organic nitrogen compound
  • Hydrocarbon derivative
  • Organic oxide
  • Primary amine
  • Organooxygen compound
  • Organonitrogen compound
  • Organopnictogen compound
  • Primary aliphatic amine
  • Organic oxygen compound
  • Carbonyl group
  • Amine
  • Aldehyde
  • Alcohol
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors
Ontology
Physiological effectNot Available
Disposition
Process
RoleNot Available
Physical Properties
StateSolid
Experimental Molecular Properties
PropertyValueReference
Melting PointNot AvailableNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Experimental Chromatographic PropertiesNot Available
Predicted Molecular Properties
Predicted Chromatographic Properties
Spectra
Biological Properties
Cellular Locations
  • Mitochondria
Biospecimen LocationsNot Available
Tissue LocationsNot Available
Pathways
Normal Concentrations
Not Available
Abnormal Concentrations
Not Available
Associated Disorders and Diseases
Disease ReferencesNone
Associated OMIM IDsNone
DrugBank IDNot Available
Phenol Explorer Compound IDNot Available
FooDB IDFDB023970
KNApSAcK IDNot Available
Chemspider ID389693
KEGG Compound IDC05938
BioCyc IDNot Available
BiGG ID1447438
Wikipedia LinkNot Available
METLIN IDNot Available
PubChem Compound440851
PDB IDNot Available
ChEBI ID27809
Food Biomarker OntologyNot Available
VMH ID4HGLUSA
MarkerDB IDNot Available
Good Scents IDNot Available
References
Synthesis ReferenceNot Available
Material Safety Data Sheet (MSDS)Not Available
General ReferencesNot Available

Enzymes

General function:
Involved in oxidoreductase activity
Specific function:
Irreversible conversion of delta-1-pyrroline-5-carboxylate (P5C), derived either from proline or ornithine, to glutamate. This is a necessary step in the pathway interconnecting the urea and tricarboxylic acid cycles. The preferred substrate is glutamic gamma-semialdehyde, other substrates include succinic, glutaric and adipic semialdehydes.
Gene Name:
ALDH4A1
Uniprot ID:
P30038
Molecular weight:
55117.24
Reactions
4-Hydroxy-L-glutamic acid + NADH + Hydrogen Ion → L-4-Hydroxyglutamate semialdehyde + NAD + Waterdetails
General function:
Involved in proline dehydrogenase activity
Specific function:
Converts proline to delta-1-pyrroline-5-carboxylate.
Gene Name:
PRODH
Uniprot ID:
O43272
Molecular weight:
56196.675
Reactions
4-Hydroxy-L-glutamic acid + NADH + Hydrogen Ion → L-4-Hydroxyglutamate semialdehyde + NAD + Waterdetails