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Record Information
Version2.0
Creation Date2014-10-02 18:58:05 UTC
Update Date2018-03-21 17:46:19 UTC
Accession NumberT3D4962
Identification
Common NameGalactosylsphingosine
ClassSmall Molecule
DescriptionGalactosylsphingosine (also known as psychosine), is an intermediate in the biosynthesis of cerebrosides. It is formed from the reaction of sphingosine with UDP-galactose and then reacts with fatty acid-coenzyme A to form the cerebroside. It is a galactoside metabolite of sphingosine and can function as a neurotoxin and a metabotoxin. A neurotoxin is a compound that disrupts or attacks neural cells and neural tissue. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. Chronically high levels of galactosylsphingosine are associated with globoid cell leukodystrophy (Krabbe disease), which is characterized by the dysfunction of galactosylceramidase. Galactosylsphingosine is a highly cytotoxic lipid capable of inducing cell death in a wide variety of cell types including oligodendrocytes. It is known to accumulate in the nervous system in the absence of galactosylceramidase. Galactosylsphingosine localizes to lipid rafts and perturbs membrane integrity. It also inhibits protein kinase C translocation to the plasma membrane (PMID: 24006512). Symptoms of Krabbe disease begin between the ages of 3 and 6 months with irritability, fevers, limb stiffness, seizures, feeding difficulties, vomiting, and slowing of mental and motor development. In the first stages of the disease, the symptoms are often mistaken with those of cerebral palsy. Other symptoms include muscle weakness, spasticity, deafness, optic atrophy, optic nerve enlargement, blindness, paralysis, and difficulty when swallowing.
Compound Type
  • Animal Toxin
  • Metabolite
  • Natural Compound
Chemical Structure
Thumb
Synonyms
Synonym
(E)-DL-erythro-b-D-2-amino-3-hydroxy-4-octadecenyl Galactopyranoside
(E)-DL-erythro-beta-delta-2-amino-3-hydroxy-4-octadecenyl Galactopyranoside
2-Amino-3-hydroxy-4-octadecenyl Galactopyranoside
Erythro-Psychosine
Psychosine
Sphingosine galactoside
[R-[R*,S*-(E)]]-2-amino-3-hydroxy-4-octadecenyl b-D-Galactopyranoside
[R-[R*,S*-(E)]]-2-amino-3-hydroxy-4-octadecenyl b-delta-Galactopyranoside
Chemical FormulaC24H47NO7
Average Molecular Mass461.633 g/mol
Monoisotopic Mass461.335 g/mol
CAS Registry Number2238-90-6
IUPAC Name(3R,4R,5R,6R)-2-{[(4E)-2-amino-3-hydroxyoctadec-4-en-1-yl]oxy}-6-(hydroxymethyl)oxane-3,4,5-triol
Traditional Name(3R,4R,5R,6R)-2-{[(4E)-2-amino-3-hydroxyoctadec-4-en-1-yl]oxy}-6-(hydroxymethyl)oxane-3,4,5-triol
SMILESCCCCCCCCCCCCC\C=C\C(O)C(N)COC1O[C@H](CO)[C@H](O)[C@@H](O)[C@H]1O
InChI IdentifierInChI=1S/C24H47NO7/c1-2-3-4-5-6-7-8-9-10-11-12-13-14-15-19(27)18(25)17-31-24-23(30)22(29)21(28)20(16-26)32-24/h14-15,18-24,26-30H,2-13,16-17,25H2,1H3/b15-14+/t18?,19?,20-,21+,22-,23-,24?/m1/s1
InChI KeyInChIKey=HHJTWTPUPVQKNA-LUIGAKQRSA-N
Chemical Taxonomy
Description belongs to the class of organic compounds known as glycosphingolipids. These are sphingolipids containing a saccharide moiety glycosidically attached to the sphingoid base. Although saccharide moieties are mostly O-glycosidically linked to the ceramide moiety, other sphingolipids with glycosidic bonds of other types (e.g. S-,C-, or N-type) has been reported.
KingdomOrganic compounds
Super ClassLipids and lipid-like molecules
ClassSphingolipids
Sub ClassGlycosphingolipids
Direct ParentGlycosphingolipids
Alternative Parents
Substituents
  • Glycosphingolipid
  • Fatty acyl glycoside of mono- or disaccharide
  • Fatty acyl glycoside
  • Hexose monosaccharide
  • Alkyl glycoside
  • O-glycosyl compound
  • Glycosyl compound
  • Fatty acyl
  • Oxane
  • Monosaccharide
  • Secondary alcohol
  • Oxacycle
  • Organoheterocyclic compound
  • Polyol
  • Acetal
  • Organic nitrogen compound
  • Organic oxygen compound
  • Organopnictogen compound
  • Hydrocarbon derivative
  • Primary amine
  • Primary alcohol
  • Organooxygen compound
  • Organonitrogen compound
  • Primary aliphatic amine
  • Amine
  • Alcohol
  • Aliphatic heteromonocyclic compound
Molecular FrameworkAliphatic heteromonocyclic compounds
External DescriptorsNot Available
Biological Properties
StatusDetected and Not Quantified
OriginNot Available
Cellular Locations
  • Extracellular
  • Membrane
Biofluid LocationsNot Available
Tissue Locations
  • Brain
  • Fibroblasts
  • Myelin
  • Nervous Tissues
PathwaysNot Available
ApplicationsNot Available
Biological RolesNot Available
Chemical RolesNot Available
Physical Properties
StateSolid
AppearanceWhite powder.
Experimental Properties
PropertyValue
Melting PointNot Available
Boiling PointNot Available
SolubilityNot Available
LogPNot Available
Predicted Properties
PropertyValueSource
Water Solubility0.09 g/LALOGPS
logP3.16ALOGPS
logP2.8ChemAxon
logS-3.7ALOGPS
pKa (Strongest Acidic)12.21ChemAxon
pKa (Strongest Basic)9.12ChemAxon
Physiological Charge1ChemAxon
Hydrogen Acceptor Count8ChemAxon
Hydrogen Donor Count6ChemAxon
Polar Surface Area145.63 ŲChemAxon
Rotatable Bond Count18ChemAxon
Refractivity124.31 m³·mol⁻¹ChemAxon
Polarizability55.23 ųChemAxon
Number of Rings1ChemAxon
Bioavailability0ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectra
Spectra
Spectrum TypeDescriptionSplash KeyView
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-01r6-0130900000-7a503060b66c5017ae40JSpectraViewer
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-01q9-1491400000-e85ed6f61bafbbcfcc24JSpectraViewer
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-009y-7950000000-b8491ff49cf9391756d4JSpectraViewer
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-03di-2341900000-77f971902b923c776e2bJSpectraViewer
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-03k9-3690300000-cc6b9deb449169b31745JSpectraViewer
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-0k96-9430000000-b94f52ab89037183039aJSpectraViewer
Toxicity Profile
Route of ExposureNot Available
Mechanism of ToxicityNot Available
MetabolismNot Available
Toxicity ValuesNot Available
Lethal DoseNot Available
Carcinogenicity (IARC Classification)No indication of carcinogenicity to humans (not listed by IARC).
Uses/SourcesNot Available
Minimum Risk LevelNot Available
Health EffectsChronically high levels of psychosine are associated with Globoid Cell Leukodystrophy.
SymptomsNot Available
TreatmentNot Available
Normal Concentrations
Not Available
Abnormal Concentrations
Not Available
DrugBank IDNot Available
HMDB IDHMDB00648
PubChem Compound ID22833541
ChEMBL IDNot Available
ChemSpider ID17215986
KEGG IDC01747
UniProt IDNot Available
OMIM ID
ChEBI ID16874
BioCyc IDNot Available
CTD IDNot Available
Stitch IDNot Available
PDB IDNot Available
ACToR IDNot Available
Wikipedia LinkNot Available
References
Synthesis ReferenceNot Available
MSDSNot Available
General References
  1. Shinoda H, Kobayashi T, Katayama M, Goto I, Nagara H: Accumulation of galactosylsphingosine (psychosine) in the twitcher mouse: determination by HPLC. J Neurochem. 1987 Jul;49(1):92-9. [3585345 ]
  2. Yoshimura T, Kobayashi T, Mitsuo K, Goto I: Decreased fatty acylation of myelin proteolipid protein in the twitcher mouse. J Neurochem. 1989 Mar;52(3):836-41. [2465381 ]
  3. Igisu H, Suzuki K: Progressive accumulation of toxic metabolite in a genetic leukodystrophy. Science. 1984 May 18;224(4650):753-5. [6719111 ]
  4. Chiba M, Tsuchihashi K, Suetake K, Ibayashi Y, Gasa S, Hashi K: Photoaffinity labeling of lipoproteins in human cerebrospinal fluid with a heterobifunctional derivative of galactosylsphingosine. Biochem Mol Biol Int. 1994 Apr;32(5):961-71. [8069245 ]
  5. Kobayashi T, Goto I, Yamanaka T, Suzuki Y, Nakano T, Suzuki K: Infantile and fetal globoid cell leukodystrophy: analysis of galactosylceramide and galactosylsphingosine. Ann Neurol. 1988 Oct;24(4):517-22. [3239954 ]
  6. Kobayashi T, Shinoda H, Goto I, Yamanaka T, Suzuki Y: Globoid cell leukodystrophy is a generalized galactosylsphingosine (psychosine) storage disease. Biochem Biophys Res Commun. 1987 Apr 14;144(1):41-6. [3579916 ]
  7. Shen JS, Watabe K, Meng XL, Ida H, Ohashi T, Eto Y: Establishment and characterization of spontaneously immortalized Schwann cells from murine model of globoid cell leukodystrophy (twitcher). J Neurosci Res. 2002 Jun 1;68(5):588-94. [12111848 ]
  8. Harzer K, Knoblich R, Rolfs A, Bauer P, Eggers J: Residual galactosylsphingosine (psychosine) beta-galactosidase activities and associated GALC mutations in late and very late onset Krabbe disease. Clin Chim Acta. 2002 Mar;317(1-2):77-84. [11814461 ]
Gene Regulation
Up-Regulated GenesNot Available
Down-Regulated GenesNot Available