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Record Information
Version5.0
StatusExpected but not Quantified
Creation Date2021-02-08 17:48:36 UTC
Update Date2021-09-14 15:18:11 UTC
HMDB IDHMDB0240745
Secondary Accession NumbersNone
Metabolite Identification
Common Name4,5-dihydroxyhexanoic acid
Description4,5-dihydroxyhexanoic acid or DHHA is a water-soluble organic acid. It probably arises from the reaction of succinic semialdehyde with an intermediate in the pyruvate dehydrogenase pathway. DHHA is found in the urine and appears to be a marker for Succinic semialdehyde dehydrogenase (SSADH) deficiency (PMID: 3126356 ). SSADH is a disorder that arises from a defect in gamma-aminobutyric acid catabolism, resulting in the accumulation of gamma-hydroxybutyric acid (GHB), 2,4-dihydroxybutyric acid, adipic acid as well as elevated levels of 4,5-dihydroxyhexanoic acid, and 4,5-dihydroxyhexanoic acid lactone. SSADH causes neurological and cognitive disorders of varying severity (PMID: 3126356 ). Children with SSADH deficiency usually manifest with developmental delay, behavioral symptoms, language dysfunction, seizures, hypotonia, extrapyramidal symptoms, and ataxia (PMID: 32055132 ).
Structure
Data?1612813508
Synonyms
ValueSource
4,5-DihydroxyhexanoateGenerator
4,5-dihydroxyhexanoic acidHMDB
DHHAHMDB
Chemical FormulaC6H12O4
Average Molecular Weight148.158
Monoisotopic Molecular Weight148.073558866
IUPAC NameNot Available
Traditional NameNot Available
CAS Registry NumberNot Available
SMILES
CC(O)C(O)CCC(O)=O
InChI Identifier
InChI=1S/C6H12O4/c1-4(7)5(8)2-3-6(9)10/h4-5,7-8H,2-3H2,1H3,(H,9,10)
InChI KeyFKHCKVQMPWUKCV-UHFFFAOYSA-N
Chemical Taxonomy
Description Belongs to the class of organic compounds known as medium-chain hydroxy acids and derivatives. These are hydroxy acids with a 6 to 12 carbon atoms long side chain.
KingdomOrganic compounds
Super ClassOrganic acids and derivatives
ClassHydroxy acids and derivatives
Sub ClassMedium-chain hydroxy acids and derivatives
Direct ParentMedium-chain hydroxy acids and derivatives
Alternative Parents
Substituents
  • Medium-chain hydroxy acid
  • Medium-chain fatty acid
  • Hydroxy fatty acid
  • Fatty acyl
  • Fatty acid
  • 1,2-diol
  • Secondary alcohol
  • Carboxylic acid derivative
  • Carboxylic acid
  • Monocarboxylic acid or derivatives
  • Organic oxide
  • Alcohol
  • Organic oxygen compound
  • Carbonyl group
  • Hydrocarbon derivative
  • Organooxygen compound
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External DescriptorsNot Available
Ontology
Not Available
Physical Properties
StateNot Available
Experimental Molecular Properties
PropertyValueReference
Melting PointNot AvailableNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Predicted Molecular Properties
PropertyValueSource
Predicted Spectral Properties

Collision Cross Sections

NameAdductTypeData SourceValueReference

Retention Indices

Underivatized

Not Available

Derivatized

DerivativeValueReference
4,5-dihydroxyhexanoic acid,1TMS,#11464.787https://arxiv.org/abs/1905.12712
4,5-dihydroxyhexanoic acid,1TMS,#21457.9955https://arxiv.org/abs/1905.12712
4,5-dihydroxyhexanoic acid,1TMS,#31438.67https://arxiv.org/abs/1905.12712
4,5-dihydroxyhexanoic acid,2TMS,#11520.1006https://arxiv.org/abs/1905.12712
4,5-dihydroxyhexanoic acid,2TMS,#21490.4875https://arxiv.org/abs/1905.12712
4,5-dihydroxyhexanoic acid,2TMS,#31490.3179https://arxiv.org/abs/1905.12712
4,5-dihydroxyhexanoic acid,3TMS,#11548.6254https://arxiv.org/abs/1905.12712
4,5-dihydroxyhexanoic acid,1TBDMS,#11700.081https://arxiv.org/abs/1905.12712
4,5-dihydroxyhexanoic acid,1TBDMS,#21697.8026https://arxiv.org/abs/1905.12712
4,5-dihydroxyhexanoic acid,1TBDMS,#31669.4519https://arxiv.org/abs/1905.12712
4,5-dihydroxyhexanoic acid,2TBDMS,#11978.5828https://arxiv.org/abs/1905.12712
4,5-dihydroxyhexanoic acid,2TBDMS,#21933.3772https://arxiv.org/abs/1905.12712
4,5-dihydroxyhexanoic acid,2TBDMS,#31924.5448https://arxiv.org/abs/1905.12712
4,5-dihydroxyhexanoic acid,3TBDMS,#12178.7202https://arxiv.org/abs/1905.12712
Spectra
Biological Properties
Cellular LocationsNot Available
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 IDNot Available
KNApSAcK IDNot Available
Chemspider ID60645551
KEGG Compound IDNot Available
BioCyc IDNot Available
BiGG IDNot Available
Wikipedia LinkNot Available
METLIN IDNot Available
PubChem Compound57417380
PDB IDNot Available
ChEBI IDNot Available
Food Biomarker OntologyNot Available
VMH IDNot Available
MarkerDB IDNot Available
References
Synthesis ReferenceNot Available
Material Safety Data Sheet (MSDS)Not Available
General References
  1. Brown GK, Cromby CH, Manning NJ, Pollitt RJ: Urinary organic acids in succinic semialdehyde dehydrogenase deficiency: evidence of alpha-oxidation of 4-hydroxybutyric acid, interaction of succinic semialdehyde with pyruvate dehydrogenase and possible secondary inhibition of mitochondrial beta-oxidation. J Inherit Metab Dis. 1987;10(4):367-75. [PubMed:3126356 ]
  2. Yoganathan S, Arunachal G, Kratz L, Varman M, Thomas M, Sudhakar SV, Oommen SP, Danda S: Metabolic Stroke: A Novel Presentation in a Child with Succinic Semialdehyde Dehydrogenase Deficiency. Ann Indian Acad Neurol. 2020 Jan-Feb;23(1):113-117. doi: 10.4103/aian.AIAN_213_18. [PubMed:32055132 ]