| Record Information |
|---|
| Version |
3.5 |
| Creation Date |
2008-09-26 08:11:50 -0600 |
| Update Date |
2013-02-08 17:25:37 -0700 |
| HMDB ID |
HMDB10714 |
| Secondary Accession Numbers |
None |
| Metabolite Identification |
|---|
| Common Name |
Galactosylceramide (d18:1/18:1(9Z)) |
| Description |
Galactosylceramide (GalCer) is a non-acidic monoglycosphingolipid, i.e. a sphingolipid with one carbohydrate moiety attached to a ceramide unit. It is an intermediate in sphingolipid metabolism and is the second to last step in the synthesis of digalactosylceramidesulfate. GalCer is generated from ceramide via the enzyme UDP-galactose ceramide galactosyltransferase [EC:2.4.1.47]. It can be converted to digalactosylceramide via the enzyme glycosyltransferases [EC 2.4.1.-]. Galactosylceramide is the principal glycosphingolipid in brain tissue, hence the trivial name "cerebroside", which was first conferred on it in 1874. Galactosylceramides are found in all nervous tissues, but they can amount to 2% of the dry weight of grey matter and 12% of white matter. They are major constituents of oligodendrocytes. Synthesis of galactosylceramide takes place on the lumenal surface of the endoplasmic reticulum, although it has free access to the cytosolic surface by an energy-independent flip-flop process. GalCer sits in the extracellular leaflet of cell membranes in nanometer sized domains or rafts. The local clustering of GalCer within rafts is thought to facilitate the initial adhesion of certain viruses, including HIV-1 and bacteria to cells through multivalent interactions between receptor proteins and GalCer. A defect in the degradation of cerbrosides leads to a disorder called Krabbe disease. Krabbe disease (also known as globoid cell leukodystrophy or galactosylceramide lipidosis) is a rare, often fatal degenerative disorder that affects the myelin sheath of the nervous system. Krabbe disease is caused by mutations in the GALC gene, which causes a deficiency of galactosylceramidase. Infants with Krabbe disease are normal at birth. Symptoms 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. There are also juvenile- and adult-onset cases of Krabbe disease, which have similar symptoms but slower progression. In infants, the disease is generally fatal before age 2. Patients with late-onset Krabbe disease tend to have a slower progression of the disease and live significantly longer.Cerebrosides are glycosphingolipids. There are four types of glycosphingolipids, the cerebrosides, sulfatides, globosides and gangliosides. Cerebrosides have a single sugar group linked to ceramide. The most common are galactocerebrosides (containing galactose), the least common are glucocerebrosides (containing glucose). Galactocerebrosides are found predominantly in neuronal cell membranes. In contrast glucocerebrosides are not normally found in membranes. Instead, they are typically intermediates in the synthesis or degradation of more complex glycosphingolipids. Galactocerebrosides are synthesized from ceramide and UDP-galactose. Excess lysosomal accumulation of glucocerebrosides is found in Gaucher disease. |
| Structure |
Download:
MOL |
SDF |
SMILES |
InChI
Display:
2D Structure |
3D Structure
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| Synonyms |
- a-GalCer
- alpha-GalCer
- Cerebroside
- D-Galactosyl-N-acylsphingosine
- D-Galactosylceramide
- delta-Galactosyl-N-acylsphingosine
- delta-Galactosylceramide
- Gal-b-Cer
- Gal-beta-1-1'Cer
- Gal-beta-Cer
- Galactocerebroside
- Galactosylceramide
- GalCer
- N-(9Z-Octadecenoyl)-1-b-galactosyl-sphing-4-enine
- N-(9Z-Octadecenoyl)-1-beta-galactosyl-sphing-4-enine
|
| Chemical Formula |
C42H79NO8 |
| Average Molecular Weight |
726.0786 |
| Monoisotopic Molecular Weight |
725.580568509 |
| IUPAC Name |
(9Z)-N-[(4Z)-3-hydroxy-1-{[(2R,3R,4S,5R,6R)-3,4,5-trihydroxy-6-(hydroxymethyl)oxan-2-yl]oxy}octadec-4-en-2-yl]octadec-9-enamide |
| Traditional IUPAC Name |
(9Z)-N-[(4Z)-3-hydroxy-1-{[(2R,3R,4S,5R,6R)-3,4,5-trihydroxy-6-(hydroxymethyl)oxan-2-yl]oxy}octadec-4-en-2-yl]octadec-9-enamide |
| CAS Registry Number |
Not Available |
| SMILES |
CCCCCCCCCCCCC\C=C/C(O)C(CO[C@@H]1O[C@H](CO)[C@H](O)[C@H](O)[C@H]1O)NC(=O)CCCCCCC\C=C/CCCCCCCC |
| InChI Identifier |
InChI=1S/C42H79NO8/c1-3-5-7-9-11-13-15-17-18-20-22-24-26-28-30-32-38(46)43-35(34-50-42-41(49)40(48)39(47)37(33-44)51-42)36(45)31-29-27-25-23-21-19-16-14-12-10-8-6-4-2/h17-18,29,31,35-37,39-42,44-45,47-49H,3-16,19-28,30,32-34H2,1-2H3,(H,43,46)/b18-17-,31-29-/t35?,36?,37-,39+,40+,41-,42-/m1/s1 |
| InChI Key |
MVGFIPNJBNBHNC-XJPHKABUSA-N |
| Chemical Taxonomy |
|---|
| Kingdom |
Organic Compounds |
| Super Class |
Lipids |
| Class |
Sphingolipids |
| Sub Class |
Neutral Glycosphingolipids |
| Other Descriptors |
- Aliphatic Heteromonocyclic Compounds
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| Substituents |
- 1,2 Diol
- Acetal
- Alkyl Glycoside
- Allyl Alcohol
- Carboxamide Group
- Ceramide
- Fatty Acyl Glycoside
- Glycosyl Compound
- Hexose Monosaccharide
- N Acyl Amine
- O Glycosyl Compound
- Oxane
- Primary Alcohol
- Saccharide
- Secondary Alcohol
- Secondary Carboxylic Acid Amide
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| Direct Parent |
Simple Glycosylceramides |
| Ontology |
|---|
| Status |
Expected and Not Quantified |
| Origin |
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| Biofunction |
- Cell signaling
- Energy source
- Fuel and energy storage
- Fuel or energy source
- Membrane component
- Membrane integrity/stability
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| Application |
- Nutrients
- Stabilizers
- Surfactants and Emulsifiers
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| Cellular locations |
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| 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 |
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| Predicted Properties |
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| Spectra |
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Not Available
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| Biological Properties |
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| Cellular Locations |
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| Biofluid Locations |
Not Available
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| Tissue Location |
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| Pathways |
Not Available
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| Normal Concentrations |
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Not Available |
| Abnormal Concentrations |
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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 |
| Phenol Explorer Metabolite ID |
Not Available |
| FoodDB ID |
FDB027864 |
| KNApSAcK ID |
Not Available |
| Chemspider ID |
24765750  |
| KEGG Compound ID |
C02686 |
| BioCyc ID |
Not Available |
| BiGG ID |
Not Available |
| Wikipedia Link |
Not Available |
| NuGOwiki Link |
HMDB10714 |
| Metagene Link |
HMDB10714 |
| METLIN ID |
Not Available |
| PubChem Compound |
53480658 |
| PDB ID |
Not Available |
| ChEBI ID |
Not Available |
| References |
|---|
| Synthesis Reference |
Not Available |
| Material Safety Data Sheet (MSDS) |
Not Available
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| General References |
Not Available
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| Enzymes |
|---|
| Name: |
Galactocerebrosidase
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| Reactions: |
- D-galactosyl-N-acylsphingosine + H2O = D-galactose + N-acylsphingosine [RN:R03617]
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| Gene Name: |
GALC |
| Uniprot ID: |
P54803 |
| Protein Sequence: |
FASTA |
| Gene Sequence: |
FASTA |
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| Name: |
Sialidase-2
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| Reactions: |
- Hydrolysis of alpha-(2->3)-, alpha-(2->6)-, alpha-(2->8)- glycosidic linkages of terminal sialic acid residues in oligosaccharides, glycoproteins, glycolipids, colominic acid and synthetic substrates ALL_REAC (other) R03491 R04018 R04634 R04650 R05115 R05117 R05996(G) R05998(G) R05999(G) R06012(G) R06147(G) R06253(G) INHIBITOR 2-Deoxy-2,3-dehydro-N-acetylneuraminic acid [CPD:C04580]
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| Gene Name: |
NEU2 |
| Uniprot ID: |
Q9Y3R4 |
| Protein Sequence: |
FASTA |
| Gene Sequence: |
FASTA |
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| Name: |
Galactosylceramide sulfotransferase
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| Reactions: |
- 3'-phosphoadenylyl sulfate + a galactosylceramide = adenosine 3',5'-bisphosphate + a galactosylceramidesulfate [RN:R04017 R06279]
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| Gene Name: |
GAL3ST1 |
| Uniprot ID: |
Q99999 |
| Protein Sequence: |
FASTA |
| Gene Sequence: |
FASTA |
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| Name: |
Arylsulfatase A
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| Reactions: |
- a cerebroside 3-sulfate + H2O = a cerebroside + sulfate [RN:R04856 R06280]
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| Gene Name: |
ARSA |
| Uniprot ID: |
P15289 |
| Protein Sequence: |
FASTA |
| Gene Sequence: |
FASTA |
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| Name: |
Sialidase-4
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| Reactions: |
- Hydrolysis of alpha-(2->3)-, alpha-(2->6)-, alpha-(2->8)- glycosidic linkages of terminal sialic acid residues in oligosaccharides, glycoproteins, glycolipids, colominic acid and synthetic substrates ALL_REAC (other) R03491 R04018 R04634 R04650 R05115 R05117 R05996(G) R05998(G) R05999(G) R06012(G) R06147(G) R06253(G) INHIBITOR 2-Deoxy-2,3-dehydro-N-acetylneuraminic acid [CPD:C04580]
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| Gene Name: |
NEU4 |
| Uniprot ID: |
Q8WWR8 |
| Protein Sequence: |
FASTA |
| Gene Sequence: |
FASTA |
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| Name: |
Alpha-galactosidase A
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| Reactions: |
- Hydrolysis of terminal, non-reducing alpha-D-galactose residues in alpha-D-galactosides, including galactose oligosaccharides, galactomannans and galactolipids ALL_REAC (other) R01101 R01103 R01104 R01194 R01329 R02926 R03618 R03634 R04019 R04470 R05549 R05961(G) R06070(G) R06091(G) R06093(G) R06094(G) R06096(G) R06142(G) R06152(G)
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| Gene Name: |
GLA |
| Uniprot ID: |
P06280 |
| Protein Sequence: |
FASTA |
| Gene Sequence: |
FASTA |
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| Name: |
Lactosylceramide 4-alpha-galactosyltransferase
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| Reactions: |
- UDP-galactose + beta-D-galactosyl-(1->4)-D-glucosyl-(11)-ceramide = UDP + alpha-D-galactosyl-(1->4)-beta-D-galactosyl-(1->4)-D-glucosyl-(11)- ceramide [RN:R03486 R05960]
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| Gene Name: |
A4GALT |
| Uniprot ID: |
Q9NPC4 |
| Protein Sequence: |
FASTA |
| Gene Sequence: |
FASTA |
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| Name: |
Acid ceramidase
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| Reactions: |
- N-acylsphingosine + H2O = a carboxylate + sphingosine [RN:R01493]
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| Gene Name: |
ASAH1 |
| Uniprot ID: |
Q13510 |
| Protein Sequence: |
FASTA |
| Gene Sequence: |
FASTA |
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| Name: |
Glucosylceramidase
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| Reactions: |
- D-glucosyl-N-acylsphingosine + H2O = D-glucose + N-acylsphingosine [RN:R01498]
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| Gene Name: |
GBA |
| Uniprot ID: |
P04062 |
| Protein Sequence: |
FASTA |
| Gene Sequence: |
FASTA |
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| Name: |
Alkaline ceramidase 2
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| Reactions: |
- N-acylsphingosine + H2O = a carboxylate + sphingosine [RN:R01493]
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| Gene Name: |
ACER2 |
| Uniprot ID: |
Q5QJU3 |
| Protein Sequence: |
FASTA |
| Gene Sequence: |
FASTA |
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| Name: |
Neutral ceramidase
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| Reactions: |
- N-acylsphingosine + H2O = a carboxylate + sphingosine [RN:R01493]
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| Gene Name: |
ASAH2 |
| Uniprot ID: |
Q9NR71 |
| Protein Sequence: |
FASTA |
| Gene Sequence: |
FASTA |
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| Name: |
Alkaline ceramidase 1
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| Reactions: |
- N-acylsphingosine + H2O = a carboxylate + sphingosine [RN:R01493]
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| Gene Name: |
ACER1 |
| Uniprot ID: |
Q8TDN7 |
| Protein Sequence: |
FASTA |
| Gene Sequence: |
FASTA |
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