Human Metabolome Database: Showing metabocard for Galactosylsphingosine (HMDB0000648)

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Identification Taxonomy Ontology Physical properties Spectra Biological properties Concentrations Links References enzymes (45) Show 45 proteins XML

enzymes (45) Show 45 proteins

Record Information

Version

4.0

Status

Expected but not Quantified

Creation Date

2005-11-16 15:48:42 UTC

Update Date

2020-02-26 21:22:26 UTC

HMDB ID

HMDB0000648

Secondary Accession Numbers

HMDB00648

Metabolite Identification

Common Name

Galactosylsphingosine

Description

Galactosylsphingosine (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

MOL SDF PDB SMILES InChI

Synonyms

Value	Source
(2S,3R,4E)-2-Amino-1-(beta-D-galactopyranosyloxy)-3-hydroxyoctadec-4-ene	ChEBI
1-beta-D-Galactosphingosine	ChEBI
1-beta-D-Galactosylsphingosine	ChEBI
1-O-beta-D-Galactopyranosylsphingosine	ChEBI
1-O-beta-D-Galactosylsphingosine	ChEBI
beta-Psychosine	ChEBI
O-Galactosylsphingosine	ChEBI
Sphingosine galactoside	ChEBI
(2S,3R,4E)-2-Amino-1-(b-D-galactopyranosyloxy)-3-hydroxyoctadec-4-ene	Generator
(2S,3R,4E)-2-Amino-1-(β-D-galactopyranosyloxy)-3-hydroxyoctadec-4-ene	Generator
1-b-D-Galactosphingosine	Generator
1-b-D-Galactosylsphingosine	Generator
1-O-b-D-Galactopyranosylsphingosine	Generator
1-O-b-D-Galactosylsphingosine	Generator
b-Psychosine	Generator
Galactoside, sphingosine	HMDB
Psychosine	HMDB
Erythro-psychosine	HMDB
Galactosylsphingosine	ChEBI

Chemical Formula

C₂₄H₄₇NO₇

Average Molecular Weight

461.64

Monoisotopic Molecular Weight

461.335252857

IUPAC Name

(2R,3R,4S,5R,6R)-2-{[(4E)-2-amino-3-hydroxyoctadec-4-en-1-yl]oxy}-6-(hydroxymethyl)oxane-3,4,5-triol

Traditional Name

(2R,3R,4S,5R,6R)-2-{[(4E)-2-amino-3-hydroxyoctadec-4-en-1-yl]oxy}-6-(hydroxymethyl)oxane-3,4,5-triol

CAS Registry Number

2238-90-6

SMILES

CCCCCCCCCCCCC\C=C\[C@@H](O)[C@@H](N)CO[C@@H]1O[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+/m0/s1

InChI Key

HHJTWTPUPVQKNA-PIIMIWFASA-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.

Kingdom

Organic compounds

Super Class

Lipids and lipid-like molecules

Class

Sphingolipids

Sub Class

Glycosphingolipids

Direct Parent

Glycosphingolipids

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 Framework

Aliphatic heteromonocyclic compounds

External Descriptors

glycosylsphingoid (CHEBI:16874 )
Sphingoid base analogs (C01747 )
Basic glycosphingolipids (C01747 )

Ontology

Physiological effect

Organoleptic effect:

Touch:

Smooth

Disposition

Route of exposure:

Enteral:

Ingestion

Source:

Biological:

Animal

Biological location:

Tissue and substructures:

Nervous tissue

Organ and components:

Brain

Cell and elements:

Extracellular

Cell:

Fibroblast

Subcellular:

Process

Naturally occurring process:

Biological process:

Chemical reaction:

Lipid peroxidation

Biochemical pathway:

Lipid metabolism pathway

Biochemical process:

Cellular process:

Cell signaling

Role

Indirect biological role:

Industrial application:

Food and nutrition:

Biological role:

Molecular messenger:

Signaling molecule

Physical Properties

State

Solid

Experimental Properties

Property	Value	Reference
Melting Point	Not Available	Not Available
Boiling Point	Not Available	Not Available
Water Solubility	Not Available	Not Available
LogP	Not Available	Not Available

Predicted Properties

Property	Value	Source
Water Solubility	0.09 g/L	ALOGPS
logP	3.16	ALOGPS
logP	2.8	ChemAxon
logS	-3.7	ALOGPS
pKa (Strongest Acidic)	12.21	ChemAxon
pKa (Strongest Basic)	9.12	ChemAxon
Physiological Charge	1	ChemAxon
Hydrogen Acceptor Count	8	ChemAxon
Hydrogen Donor Count	6	ChemAxon
Polar Surface Area	145.63 Å²	ChemAxon
Rotatable Bond Count	18	ChemAxon
Refractivity	124.31 m³·mol⁻¹	ChemAxon
Polarizability	54.48 Å³	ChemAxon
Number of Rings	1	ChemAxon
Bioavailability	No	ChemAxon
Rule of Five	No	ChemAxon
Ghose Filter	No	ChemAxon
Veber's Rule	No	ChemAxon
MDDR-like Rule	No	ChemAxon

Spectra

Not Available

Biological Properties

Cellular Locations

Extracellular
Membrane (predicted from logP)

Biospecimen Locations

Not Available

Tissue Locations

Brain
Fibroblasts
Myelin
Nervous Tissues

Pathways

Not Available

Name	SMPDB/Pathwhiz	KEGG

Normal Concentrations

Not Available

Abnormal Concentrations

Not Available

Associated Disorders and Diseases

Disease References

None

Associated OMIM IDs

None

External Links

DrugBank ID

Not Available

Phenol Explorer Compound ID

Not Available

FoodDB ID

FDB022161

KNApSAcK ID

Not Available

Chemspider ID

4444111

KEGG Compound ID

C01747

BioCyc ID

PSYCHOSINE

BiGG ID

Not Available

Wikipedia Link

Psychosine

METLIN ID

5620

PubChem Compound

5280458

PDB ID

Not Available

ChEBI ID

16874

Food Biomarker Ontology

Not Available

VMH ID

C01747

References

Synthesis Reference

Shapiro, 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

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 ]
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 ]
Igisu H, Suzuki K: Progressive accumulation of toxic metabolite in a genetic leukodystrophy. Science. 1984 May 18;224(4650):753-5. [PubMed:6719111 ]
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 ]
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 ]
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 ]
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 ]
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 ]
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.

Show all enzymes and transporters

Enzymes

Enzyme Details

1. Galactocerebrosidase

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

Enzyme Details

2. Galactosylceramide sulfotransferase

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

Enzyme Details

3. Globoside alpha-1,3-N-acetylgalactosaminyltransferase 1

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

Enzyme Details

4. N-acetylglucosaminyl-phosphatidylinositol de-N-acetylase

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

Enzyme Details

5. Lactosylceramide 4-alpha-galactosyltransferase

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

Enzyme Details

6. Phosphatidylinositol N-acetylglucosaminyltransferase subunit Q

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

Enzyme Details

7. Phosphatidylinositol N-acetylglucosaminyltransferase subunit A

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

Enzyme Details

8. Phosphatidylinositol N-acetylglucosaminyltransferase subunit H

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

Enzyme Details

9. Phosphatidylinositol N-acetylglucosaminyltransferase subunit P

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

Enzyme Details

10. Phosphatidylinositol N-acetylglucosaminyltransferase subunit C

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.

Show all enzymes and transporters

Showing metabocard for Galactosylsphingosine (HMDB0000648)

Structure for HMDB0000648 (Galactosylsphingosine)

Enzymes