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Record Information
Version5.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2023-02-21 17:14:57 UTC
HMDB IDHMDB0000565
Secondary Accession Numbers
  • HMDB00565
Metabolite Identification
Common NameGalactonic acid
DescriptionGalactonic acid is a sugar acid that is a metabolic breakdown product of galactose. Galactose dehydrogenase is responsible for converting galactose to galactonolactone, which then spontaneously or enzymatically converts to galactonic acid. Once formed, galactonic acid may enter the pentose phosphate pathway. Galactonic acid is increased in red blood cells of galactosemic patients, due to a galactose-1-phosphate uridyltransferase (GALT) deficiency (PMID: 14680973 , OMMBID: The Online Metabolic and Molecular Bases of Inherited Disease, Ch.72). When present in sufficiently high levels, galactonic acid can act as an acidogen and a metabotoxin. An acidogen is an acidic compound that induces acidosis, which has multiple adverse effects on many organ systems. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. Chronically high levels of galactonic acid are associated with at least two inborn errors of metabolism, including galactosemia and galactosemia type II. Galactonic acid is an organic acid. Abnormally high levels of organic acids in the blood (organic acidemia), urine (organic aciduria), the brain, and other tissues lead to general metabolic acidosis. Acidosis typically occurs when arterial pH falls below 7.35. In infants with acidosis, the initial symptoms include poor feeding, vomiting, loss of appetite, weak muscle tone (hypotonia), and lack of energy (lethargy). These can progress to heart abnormalities, liver abnormalities (jaundice), kidney abnormalities, seizures, coma, and possibly death. These are also the characteristic symptoms of untreated galactosemia. Many affected children with organic acidemias experience intellectual disability or delayed development. High levels of galactonic acid in infants are specifically associated with hepatomegaly (an enlarged liver), cirrhosis, renal failure, cataracts, vomiting, seizure, hypoglycemia, lethargy, brain damage, and ovarian failure.
Structure
Thumb
Synonyms
ValueSource
D-Galactonic acidKegg
D-GalactonateGenerator
GalactonateGenerator
Galactonic acid, (D)-isomerHMDB
Galactonic acid, monopotassium saltHMDB
Galactonic acid, (L)-isomerHMDB
Chemical FormulaC6H12O7
Average Molecular Weight196.1553
Monoisotopic Molecular Weight196.058302738
IUPAC Name(2R,3S,4S,5R)-2,3,4,5,6-pentahydroxyhexanoic acid
Traditional Namegalactonic acid
CAS Registry Number13382-27-9
SMILES
OC[C@@H](O)[C@H](O)[C@H](O)[C@@H](O)C(O)=O
InChI Identifier
InChI=1S/C6H12O7/c7-1-2(8)3(9)4(10)5(11)6(12)13/h2-5,7-11H,1H2,(H,12,13)/t2-,3+,4+,5-/m1/s1
InChI KeyRGHNJXZEOKUKBD-MGCNEYSASA-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
  • Beta-hydroxy acid
  • Hydroxy fatty acid
  • Alpha-hydroxy acid
  • Monosaccharide
  • Fatty acyl
  • Fatty acid
  • Secondary alcohol
  • Polyol
  • Monocarboxylic acid or derivatives
  • Carboxylic acid
  • Carboxylic acid derivative
  • Organooxygen compound
  • Hydrocarbon derivative
  • Organic oxide
  • Carbonyl group
  • Primary alcohol
  • Alcohol
  • Organic oxygen compound
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors
Ontology
Physiological effect
Disposition
Process
Role
Physical Properties
StateSolid
Experimental Molecular Properties
PropertyValueReference
Melting Point133 - 136 °CNot 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
  • Cytoplasm
Biospecimen Locations
  • Blood
  • Feces
  • Urine
Tissue Locations
  • Erythrocyte
Pathways
Normal Concentrations
Abnormal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified3.00 (0.68 - 6.47) uMChildren (1-13 years old)Not Specified
Galactosemia
details
UrineDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothBladder cancer details
Associated Disorders and Diseases
Disease References
Galactosemia
  1. Yager CT, Chen J, Reynolds R, Segal S: Galactitol and galactonate in red blood cells of galactosemic patients. Mol Genet Metab. 2003 Nov;80(3):283-9. [PubMed:14680973 ]
Associated OMIM IDsNone
DrugBank IDNot Available
Phenol Explorer Compound IDNot Available
FooDB IDFDB022122
KNApSAcK IDC00053191
Chemspider ID114198
KEGG Compound IDC00880
BioCyc IDNot Available
BiGG IDNot Available
Wikipedia LinkNot Available
METLIN ID3336
PubChem Compound128869
PDB IDNot Available
ChEBI ID16534
Food Biomarker OntologyNot Available
VMH IDGALCTN_D
MarkerDB IDNot Available
Good Scents IDNot Available
References
Synthesis ReferenceLevene, P. A.; Simms, H. S. Lactone formation mono- and dicarboxylic sugar acids. Journal of Biological Chemistry (1925), 65 31-47.
Material Safety Data Sheet (MSDS)Not Available
General References
  1. Shoemaker JD, Elliott WH: Automated screening of urine samples for carbohydrates, organic and amino acids after treatment with urease. J Chromatogr. 1991 Jan 2;562(1-2):125-38. [PubMed:2026685 ]
  2. Shinka T, Inoue Y, Peng H, Zhen-Wei X, Ose M, Kuhara T: Urine screening of five-day-old newborns: metabolic profiling of neonatal galactosuria. J Chromatogr B Biomed Sci Appl. 1999 Sep 24;732(2):469-77. [PubMed:10517369 ]
  3. Yager CT, Chen J, Reynolds R, Segal S: Galactitol and galactonate in red blood cells of galactosemic patients. Mol Genet Metab. 2003 Nov;80(3):283-9. [PubMed:14680973 ]