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Record Information
Version4.0
StatusExpected but not Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2018-03-05 09:12:08 UTC
HMDB IDHMDB0000648
Secondary Accession Numbers
  • HMDB00648
Metabolite Identification
Common NameGalactosylsphingosine
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.
Structure
Thumb
Synonyms
ValueSource
(e)-DL-erythro-b-D-2-amino-3-Hydroxy-4-octadecenyl galactopyranosideHMDB
(e)-DL-erythro-beta-delta-2-amino-3-Hydroxy-4-octadecenyl galactopyranosideHMDB
2-amino-3-Hydroxy-4-octadecenyl galactopyranosideHMDB
erythro-PsychosineHMDB
PsychosineHMDB
Sphingosine galactosideHMDB
[R-[R*,s*-(e)]]-2-amino-3-hydroxy-4-octadecenyl b-D-galactopyranosideHMDB
[R-[R*,s*-(e)]]-2-amino-3-hydroxy-4-octadecenyl b-delta-galactopyranosideHMDB
Galactoside, sphingosineMeSH
Chemical FormulaC24H47NO7
Average Molecular Weight461.6325
Monoisotopic Molecular Weight461.335252863
IUPAC Name(3R,4S,5R,6R)-2-{[(4E)-2-amino-3-hydroxyoctadec-4-en-1-yl]oxy}-6-(hydroxymethyl)oxane-3,4,5-triol
Traditional Name(3R,4S,5R,6R)-2-{[(4E)-2-amino-3-hydroxyoctadec-4-en-1-yl]oxy}-6-(hydroxymethyl)oxane-3,4,5-triol
CAS Registry Number2238-90-6
SMILES
CCCCCCCCCCCCC\C=C\C(O)C(N)COC1O[C@H](CO)[C@H](O)[C@H](O)[C@H]1O
InChI Identifier
InChI=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 KeyHHJTWTPUPVQKNA-DWSXXLTBSA-N
Chemical Taxonomy
DescriptionThis compound 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
  • Fatty acyl glycoside of mono- or disaccharide
  • Alkyl glycoside
  • Hexose monosaccharide
  • Glycosyl compound
  • O-glycosyl compound
  • Fatty acyl
  • Oxane
  • Monosaccharide
  • Secondary alcohol
  • Oxacycle
  • Organoheterocyclic compound
  • Acetal
  • Polyol
  • Organic oxygen compound
  • Organonitrogen compound
  • Organooxygen compound
  • Primary aliphatic amine
  • Primary alcohol
  • Primary amine
  • Alcohol
  • Organic nitrogen compound
  • Amine
  • Hydrocarbon derivative
  • Organopnictogen compound
  • Aliphatic heteromonocyclic compound
Molecular FrameworkAliphatic heteromonocyclic compounds
External DescriptorsNot Available
Ontology
Physiological effect

Organoleptic effect:

Disposition

Route of exposure:

Source:

Biological location:

Process

Naturally occurring process:

Role

Indirect biological role:

Industrial application:

Biological role:

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 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
Polarizability54.82 ųChemAxon
Number of Rings1ChemAxon
Bioavailability0ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectra
Spectra
Spectrum TypeDescriptionSplash Key
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (3 TMS) - 70eV, Positivesplash10-03dr-2844309000-99e81ad37003fd8ecf8fView in MoNA
Biological Properties
Cellular Locations
  • Extracellular
  • Membrane (predicted from logP)
Biospecimen LocationsNot Available
Tissue Location
  • Brain
  • Fibroblasts
  • Myelin
  • Nervous Tissues
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
FoodDB IDFDB022161
KNApSAcK IDNot Available
Chemspider ID17215986
KEGG Compound IDC01747
BioCyc IDNot Available
BiGG IDNot Available
Wikipedia LinkNot Available
METLIN ID5620
PubChem Compound22833541
PDB IDNot Available
ChEBI ID16874
References
Synthesis ReferenceShapiro, David; Rachaman, Eliezer S.; Sheradsky, Tuvia. Synthetic studies on sphingolipids. X. Synthesis of psychosine. Journal of the American Chemical Society (1964), 86(20), 4472-6.
Material Safety Data Sheet (MSDS)Not 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. [PubMed: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. [PubMed:2465381 ]
  3. Igisu H, Suzuki K: Progressive accumulation of toxic metabolite in a genetic leukodystrophy. Science. 1984 May 18;224(4650):753-5. [PubMed: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. [PubMed: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. [PubMed: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. [PubMed: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. [PubMed: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. [PubMed:11814461 ]
  9. Hawkins-Salsbury JA, Parameswar AR, Jiang X, Schlesinger PH, Bongarzone E, Ory DS, Demchenko AV, Sands MS: Psychosine, the cytotoxic sphingolipid that accumulates in globoid cell leukodystrophy, alters membrane architecture. J Lipid Res. 2013 Dec;54(12):3303-11. doi: 10.1194/jlr.M039610. Epub 2013 Sep 4. [PubMed:24006512 ]

Only showing the first 10 proteins. There are 45 proteins in total.

Enzymes

General function:
Involved in galactosylceramidase activity
Specific function:
Hydrolyzes the galactose ester bonds of galactosylceramide, galactosylsphingosine, lactosylceramide, and monogalactosyldiglyceride. Enzyme with very low activity responsible for the lysosomal catabolism of galactosylceramide, a major lipid in myelin, kidney and epithelial cells of small intestine and colon.
Gene Name:
GALC
Uniprot ID:
P54803
Molecular weight:
77062.86
General function:
Involved in galactosylceramide sulfotransferase activity
Specific function:
Catalyzes the sulfation of membrane glycolipids. Seems to prefer beta-glycosides at the non-reducing termini of sugar chains attached to a lipid moiety. Catalyzes the synthesis of galactosylceramide sulfate (sulfatide), a major lipid component of the myelin sheath and of monogalactosylalkylacylglycerol sulfate (seminolipid), present in spermatocytes (By similarity). Also acts on lactosylceramide, galactosyl 1-alkyl-2-sn-glycerol and galactosyl diacylglycerol (in vitro).
Gene Name:
GAL3ST1
Uniprot ID:
Q99999
Molecular weight:
48763.63
General function:
Involved in transferase activity, transferring hexosyl groups
Specific function:
Catalyzes the formation of some glycolipid via the addition of N-acetylgalactosamine (GalNAc) in alpha-1,3-linkage to some substrate. Glycolipids probably serve for adherence of some pathogens
Gene Name:
GBGT1
Uniprot ID:
Q8N5D6
Molecular weight:
40126.9
General function:
Involved in N-acetylglucosaminylphosphatidylinositol de
Specific function:
Involved in the second step of GPI biosynthesis. De-N-acetylation of N-acetylglucosaminyl-phosphatidylinositol.
Gene Name:
PIGL
Uniprot ID:
Q9Y2B2
Molecular weight:
28530.965
General function:
Involved in galactosyltransferase activity
Specific function:
Necessary for the biosynthesis of the Pk antigen of blood histogroup P. Catalyzes the transfer of galactose to lactosylceramide and galactosylceramide. Necessary for the synthesis of the receptor for bacterial verotoxins.
Gene Name:
A4GALT
Uniprot ID:
Q9NPC4
Molecular weight:
40498.78
General function:
Involved in phosphatidylinositol N-acetylglucosaminyltransferase activity
Specific function:
Part of the complex catalyzing the transfer of N-acetylglucosamine from UDP-N-acetylglucosamine to phosphatidylinositol, the first step of GPI biosynthesis.
Gene Name:
PIGQ
Uniprot ID:
Q9BRB3
Molecular weight:
65343.25
General function:
Involved in biosynthetic process
Specific function:
Necessary for the synthesis of N-acetylglucosaminyl-phosphatidylinositol, the very early intermediate in GPI-anchor biosynthesis.
Gene Name:
PIGA
Uniprot ID:
P37287
Molecular weight:
54126.065
General function:
Involved in phosphatidylinositol N-acetylglucosaminyltr
Specific function:
Part of the complex catalyzing the transfer of N-acetylglucosamine from UDP-N-acetylglucosamine to phosphatidylinositol, the first step of GPI biosynthesis.
Gene Name:
PIGH
Uniprot ID:
Q14442
Molecular weight:
21080.415
General function:
Involved in phosphatidylinositol N-acetylglucosaminyltr
Specific function:
Part of the complex catalyzing the transfer of N-acetylglucosamine from UDP-N-acetylglucosamine to phosphatidylinositol, the first step of GPI biosynthesis.
Gene Name:
PIGP
Uniprot ID:
P57054
Molecular weight:
18089.055
General function:
Involved in phosphatidylinositol N-acetylglucosaminyltransferase activity
Specific function:
Part of the complex catalyzing the transfer of N-acetylglucosamine from UDP-N-acetylglucosamine to phosphatidylinositol, the first step of GPI biosynthesis.
Gene Name:
PIGC
Uniprot ID:
Q92535
Molecular weight:
33582.18

Only showing the first 10 proteins. There are 45 proteins in total.