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Record Information
Version3.6
Creation Date2006-08-12 22:40:07 UTC
Update Date2016-02-11 01:06:21 UTC
HMDB IDHMDB03518
Secondary Accession NumbersNone
Metabolite Identification
Common NameHomocitric acid
DescriptionHomocitric acid is a normal urinary organic acid. (PMID: 14708889 ). Homocitric acid is a citric acid analogue found as a minor metabolite in urine samples from patients with propionic acidaemia. Homocitric acid is formed by citrate synthase due to propionyl CoA carboxylase deficiency (by the citrate synthase condensation reaction of a-ketoglutarate with acetyl coenzyme A and propionyl coenzyme A). (PMID: 7850997 ).
Structure
Thumb
Synonyms
ValueSource
(R)-2-Hydroxy-1,2,4-butanetricarboxylic acidChEBI
(R)-2-Hydroxybutane-1,2,4-tricarboxylic acidChEBI
(R)-Homocitric acidChEBI
3-Hydroxy-3-carboxyadipic acidChEBI
HomocitrateChEBI
Homocitric acidChEBI
(R)-2-Hydroxy-1,2,4-butanetricarboxylateGenerator
(2R)-2-Hydroxybutane-1,2,4-tricarboxylateGenerator
(R)-2-Hydroxybutane-1,2,4-tricarboxylateGenerator
(R)-HomocitrateGenerator
3-Hydroxy-3-carboxyadipateGenerator
2-Hydroxybutane-1,2,4-tricarboxylateHMDB
Chemical FormulaC7H10O7
Average Molecular Weight206.1501
Monoisotopic Molecular Weight206.042652674
IUPAC Name(2R)-2-hydroxybutane-1,2,4-tricarboxylic acid
Traditional Name(R)-homocitric acid
CAS Registry Number3562-74-1
SMILES
OC(=O)CC[C@@](O)(CC(O)=O)C(O)=O
InChI Identifier
InChI=1S/C7H10O7/c8-4(9)1-2-7(14,6(12)13)3-5(10)11/h14H,1-3H2,(H,8,9)(H,10,11)(H,12,13)/t7-/m1/s1
InChI KeyInChIKey=XKJVEVRQMLKSMO-SSDOTTSWSA-N
Chemical Taxonomy
DescriptionThis compound belongs to the class of organic compounds known as tricarboxylic acids and derivatives. These are carboxylic acids containing exactly three carboxyl groups.
KingdomOrganic compounds
Super ClassOrganic acids and derivatives
ClassCarboxylic acids and derivatives
Sub ClassTricarboxylic acids and derivatives
Direct ParentTricarboxylic acids and derivatives
Alternative Parents
Substituents
  • Tricarboxylic acid or derivatives
  • Beta-hydroxy acid
  • Hydroxy acid
  • Alpha-hydroxy acid
  • Tertiary alcohol
  • Carboxylic acid
  • Hydrocarbon derivative
  • Organooxygen compound
  • Carbonyl group
  • Alcohol
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors
Ontology
StatusExpected but not Quantified
Origin
  • Endogenous
BiofunctionNot Available
ApplicationNot Available
Cellular locations
  • Cytoplasm (predicted from logP)
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 Solubility191.0 mg/mLALOGPS
logP-1.5ALOGPS
logP-1ChemAxon
logS-0.03ALOGPS
pKa (Strongest Acidic)3.09ChemAxon
pKa (Strongest Basic)-4.1ChemAxon
Physiological Charge-3ChemAxon
Hydrogen Acceptor Count7ChemAxon
Hydrogen Donor Count4ChemAxon
Polar Surface Area132.13 Å2ChemAxon
Rotatable Bond Count6ChemAxon
Refractivity40.38 m3·mol-1ChemAxon
Polarizability17.44 Å3ChemAxon
Number of Rings0ChemAxon
Bioavailability1ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectra
Spectra
Spectrum TypeDescriptionSplash Key
Predicted GC-MSPredicted GC-MS Spectrum - GC-MSNot Available
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, PositiveNot Available
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, PositiveNot Available
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, PositiveNot Available
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, NegativeNot Available
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, NegativeNot Available
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, NegativeNot Available
Biological Properties
Cellular Locations
  • Cytoplasm (predicted from logP)
Biofluid LocationsNot Available
Tissue LocationNot Available
PathwaysNot Available
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 IDFDB023186
KNApSAcK IDNot Available
Chemspider ID388564
KEGG Compound IDC01251
BioCyc IDHOMO-CIT
BiGG IDNot Available
Wikipedia LinkHomocitrate
NuGOwiki LinkHMDB03518
Metagene LinkHMDB03518
METLIN ID6943
PubChem Compound439459
PDB IDHCA
ChEBI ID52222
References
Synthesis ReferenceNot Available
Material Safety Data Sheet (MSDS)Not Available
General References
  1. Boulat O, Gradwohl M, Matos V, Guignard JP, Bachmann C: Organic acids in the second morning urine in a healthy Swiss paediatric population. Clin Chem Lab Med. 2003 Dec;41(12):1642-58. [14708889 ]
  2. van Rooyen JP, Mienie LJ, Erasmus E, de Wet WJ, Duran M, Wadman SK: Urinary excretion of homocitric acid and methylhomocitric acid in propionic acidaemia: minor metabolic products of the citrate synthase aldol condensation reaction. Clin Chim Acta. 1994 Oct 14;230(1):91-9. [7850997 ]

Enzymes

General function:
Involved in metabolic process
Specific function:
Catalyzes the isomerization of citrate to isocitrate via cis-aconitate (By similarity).
Gene Name:
ACO2
Uniprot ID:
Q99798
Molecular weight:
85424.745
Reactions
Homocitric acid → (Z)-But-1-ene-1,2,4-tricarboxylate + Waterdetails
General function:
Involved in metabolic process
Specific function:
Iron sensor. Binds a 4Fe-4S cluster and functions as aconitase when cellular iron levels are high. Functions as mRNA binding protein that regulates uptake, sequestration and utilization of iron when cellular iron levels are low. Binds to iron-responsive elements (IRES) in target mRNA species when iron levels are low. Binding of a 4Fe-4S cluster precludes RNA binding. Catalyzes the isomerization of citrate to isocitrate via cis-aconitate (By similarity).
Gene Name:
ACO1
Uniprot ID:
P21399
Molecular weight:
98398.14
Reactions
Homocitric acid → (Z)-But-1-ene-1,2,4-tricarboxylate + Waterdetails