| Record Information |
|---|
| Version |
3.5 |
| Creation Date |
2005-11-16 08:48:42 -0700 |
| Update Date |
2013-02-08 17:13:37 -0700 |
| HMDB ID |
HMDB04942 |
| Secondary Accession Numbers |
None |
| Metabolite Identification |
|---|
| Common Name |
Ganglioside GM2 (d18:1/24:1(15Z)) |
| Description |
Ganglioside GM2 (d18:1/24:1(15Z)) is a glycosphingolipid (ceramide and oligosaccharide)or oligoglycosylceramide with one or more sialic acids (i.e. n-acetylneuraminic acid) linked on the sugar chain. It is a component the cell plasma membrane which modulates cell signal transduction events. Gangliosides have been found to be highly important in immunology. Ganglioside GM2 carries a net-negative charge at pH 7.0 and is acidic. Gangliosides can amount to 6% of the weight of lipids from brain, but they are found at low levels in all animal tissues. G(M2) Ganglioside accumulates due to a deficiency of hexosaminidase A or B (beta-N-acetylhexosaminidase), or GM2 activator protein, resulting in Gangliosidoses. Gangliosidoses include heredity metabolic disorders that include Tay-Sachs disease and Sandhoff disease. Gangliosides GM2 is especially common in the nervous tissue of the brain. In the United States, about 1 in 27 to 1 in 30 Ashkenazi Jews is a recessive carrier. French Canadians and the Cajun community of Louisiana have an occurrence similar to the Ashkenazi Jews.Gangliosides are glycosphingolipids. There are four types of glycosphingolipids, the cerebrosides, sulfatides, globosides and gangliosides. Gangliosides are very similar to globosides except that they also contain N-acetyl neuraminic acid (NANA) in varying amounts. The specific names for the gangliosides provide information about their structure. The letter G refers to ganglioside, and the subscripts M, D, T and Q indicate that the molecule contains mono-, di-, tri and quatra-sialic acid. The numbered subscripts 1, 2 and 3 refer to the carbohydrate sequence that is attached to the ceramide. In particular, 1 stands for GalGalNAcGalGlc-ceramide, 2 stands for GalNAcGalGlc-ceramide and 3 stands for GalGlc-ceramide. Deficiencies in lysosomal enzymes that degrade the carbohydrate portions of various gangliosides are responsible for a number of lysosomal storage diseases such as Tay-Sachs disease, Sandhoff disease, and GM1 gangliosidosis. The carbohydrate portion of the ganglioside GM1 is the site of attachment of cholera toxin, the protein secreted by Vibrio cholerae. |
| Structure |
Download:
MOL |
SDF |
SMILES |
InChI
Display:
2D Structure |
3D Structure
|
| Synonyms |
- GalNAc-beta1->4(Neu5Ac-alpha2->3)Gal-beta1->4Glc-beta1->1'Cer
- GalNAc-beta1->4(Neu5Ac-alpha2->3)Gal-beta1->4Glc-beta1->1'Cer(d18:1/24:1
- Ganglioside GM2
- GM2
- II3NeuAcGgOse3Cer
- N-Acetyl-D-galactosaminyl-(N-acetylneuraminyl)-D-galactosyl-1,4-beta-D-glucosyl-N-acylsphingosine
- N-Acetyl-D-galactosaminyl-(N-acetylneuraminyl)-D-galactosyl-D-glucosylceramide
- N-Acetyl-delta-galactosaminyl-(N-acetylneuraminyl)-delta-galactosyl-1,4-beta-delta-glucosyl-N-acylsphingosine
- N-Acetyl-delta-galactosaminyl-(N-acetylneuraminyl)-delta-galactosyl-delta-glucosylceramide
- N-Acetyl-Ganglioside GM2
- N-Acetylganglioside GM2
- NeuAc-GM2
- Tay-Sachs ganglioside
|
| Chemical Formula |
C73H131N3O26 |
| Average Molecular Weight |
1466.8257 |
| Monoisotopic Molecular Weight |
1465.902081379 |
| IUPAC Name |
(2S,4S,5R)-2-{[(2S,3R,4R,5S,6R)-2-{[(2R,3S,4R,5R,6R)-4,5-dihydroxy-6-{[(2S,3R,4E)-3-hydroxy-2-[(15Z)-tetracos-15-enamido]octadec-4-en-1-yl]oxy}-2-(hydroxymethyl)oxan-3-yl]oxy}-5-{[(2S,3R,4R,5R,6R)-3-acetamido-4,5-dihydroxy-6-(hydroxymethyl)oxan-2-yl]oxy}-3-hydroxy-6-(hydroxymethyl)oxan-4-yl]oxy}-5-acetamido-4-hydroxy-6-[(1R,2R)-1,2,3-trihydroxypropyl]oxane-2-carboxylic acid |
| Traditional IUPAC Name |
(2S,4S,5R)-2-{[(2S,3R,4R,5S,6R)-2-{[(2R,3S,4R,5R,6R)-4,5-dihydroxy-6-{[(2S,3R,4E)-3-hydroxy-2-[(15Z)-tetracos-15-enamido]octadec-4-en-1-yl]oxy}-2-(hydroxymethyl)oxan-3-yl]oxy}-5-{[(2S,3R,4R,5R,6R)-3-acetamido-4,5-dihydroxy-6-(hydroxymethyl)oxan-2-yl]oxy}- |
| CAS Registry Number |
104443-57-4 |
| SMILES |
CCCCCCCCCCCCC\C=C\[C@@H](O)[C@H](CO[C@@H]1O[C@H](CO)[C@@H](O[C@@H]2O[C@H](CO)[C@H](O[C@@H]3O[C@H](CO)[C@H](O)[C@H](O)[C@H]3NC(C)=O)[C@H](O[C@@]3(C[C@H](O)[C@@H](NC(C)=O)C(O3)[C@H](O)[C@H](O)CO)C(O)=O)[C@H]2O)[C@H](O)[C@H]1O)NC(=O)CCCCCCCCCCCCC\C=C/CCCCCCCC |
| InChI Identifier |
InChI=1S/C73H131N3O26/c1-5-7-9-11-13-15-17-19-20-21-22-23-24-25-26-28-30-32-34-36-38-40-56(86)76-49(50(83)39-37-35-33-31-29-27-18-16-14-12-10-8-6-2)46-95-70-63(91)62(90)65(54(44-79)97-70)99-71-64(92)68(66(55(45-80)98-71)100-69-58(75-48(4)82)61(89)60(88)53(43-78)96-69)102-73(72(93)94)41-51(84)57(74-47(3)81)67(101-73)59(87)52(85)42-77/h19-20,37,39,49-55,57-71,77-80,83-85,87-92H,5-18,21-36,38,40-46H2,1-4H3,(H,74,81)(H,75,82)(H,76,86)(H,93,94)/b20-19-,39-37+/t49-,50+,51-,52+,53+,54+,55+,57+,58+,59+,60-,61+,62+,63+,64+,65+,66-,67?,68+,69-,70+,71-,73-/m0/s1 |
| InChI Key |
SKEFIZUMXOYBHZ-LCGNTRFZSA-N |
| Chemical Taxonomy |
|---|
| Kingdom |
Organic Compounds |
| Super Class |
Lipids |
| Class |
Sphingolipids |
| Sub Class |
Neutral Glycosphingolipids |
| Other Descriptors |
- Aliphatic Heteropolycyclic Compounds
- Gangliosides
|
| Substituents |
- 1,2 Diol
- 1,3 Aminoalcohol
- Acetal
- Alkyl Glycoside
- Allyl Alcohol
- Carboxamide Group
- Carboxylic Acid
- Ceramide
- Fatty Acyl Glycoside
- Glucosamine
- Glyco Amino Acid
- Glycosyl Compound
- Hexose Tetrasaccharide
- Mannosamine
- N Acetylneuraminic Acid
- N Acyl Amine
- Neuraminic Acid
- O Glycosyl Compound
- Oxane
- Primary Alcohol
- Pyranoid Amino Acid
- Saccharide
- Secondary Alcohol
- Secondary Carboxylic Acid Amide
- Sugar Acid
|
| Direct Parent |
Arthro and Ganglio series |
| Ontology |
|---|
| Status |
Expected and Not Quantified |
| Origin |
|
| Biofunction |
- Cell signaling
- Fuel and energy storage
- Fuel or energy source
- Membrane integrity/stability
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| Application |
- Nutrients
- Stabilizers
- Surfactants and Emulsifiers
|
| 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 |
Insoluble |
Not Available |
| LogP |
Not Available |
Not Available |
|
| Predicted Properties |
|
| Spectra |
|---|
|
Not Available
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| Biological Properties |
|---|
| 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 |
|---|
|
Not Available |
| Abnormal Concentrations |
|---|
|
Not Available |
| Associated Disorders and Diseases |
|---|
| Disease References |
None |
| Associated OMIM IDs |
None |
| External Links |
|---|
| DrugBank ID |
Not Available |
| DrugBank Metabolite ID |
Not Available |
| Phenol Explorer Compound ID |
Not Available |
| Phenol Explorer Metabolite ID |
Not Available |
| FoodDB ID |
FDB023530 |
| KNApSAcK ID |
Not Available |
| Chemspider ID |
16744928  |
| KEGG Compound ID |
C04884 |
| BioCyc ID |
CPD-1100 |
| BiGG ID |
Not Available |
| Wikipedia Link |
GM2 |
| NuGOwiki Link |
HMDB04942 |
| Metagene Link |
HMDB04942 |
| METLIN ID |
Not Available |
| PubChem Compound |
20057338 |
| PDB ID |
Not Available |
| ChEBI ID |
Not Available |
| References |
|---|
| Synthesis Reference |
Not Available |
| Material Safety Data Sheet (MSDS) |
Not Available
|
| General References |
- Okada N, Wu X, Mizokami M, Irie RF, Okada H: Human IgM monoclonal antibody to ganglioside GM2 and complement suppress virus propagation in ex vivo cultures of lymphocytes from HIV-1 infected patients. Microbiol Immunol. 1999;43(7):723-7.
Pubmed: 10529115
- De Vriese SR, Matthys C, De Henauw S, De Backer G, Dhont M, Christophe AB: Maternal and umbilical fatty acid status in relation to maternal diet. Prostaglandins Leukot Essent Fatty Acids. 2002 Dec;67(6):389-96.
Pubmed: 12468259
- Denny CA, Kasperzyk JL, Gorham KN, Bronson RT, Seyfried TN: Influence of caloric restriction on motor behavior, longevity, and brain lipid composition in Sandhoff disease mice. J Neurosci Res. 2006 May 1;83(6):1028-38.
Pubmed: 16521125
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| Enzymes |
|---|
| Name: |
Alpha-N-acetylneuraminide alpha-2,8-sialyltransferase
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| Reactions: |
| Reaction |
|
| Cytidine monophosphate N-acetylneuraminic acid + alpha-N-acetylneuraminyl-(2->3)-beta-D-galactosyl-R unknown Cytidine monophosphate + alpha-N-acetylneuraminyl-(2->8)-alpha-N-acetylneuraminyl-(2->3)-beta-D-galactosyl-R |
details |
| CMP-N-acetylneuraminate + GM3 unknown CMP + GD3 |
details |
| CMP-N-acetylneuraminate + GD3 unknown CMP + GT3 |
details |
| CMP-N-acetylneuraminate + Sialyl-3-paragloboside unknown CMP + 3',8'-LD1 |
details |
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| Gene Name: |
ST8SIA1 |
| Uniprot ID: |
Q92185 |
| Protein Sequence: |
FASTA |
| Gene Sequence: |
FASTA |
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| Name: |
Sialidase-2
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| Reactions: |
| Reaction |
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| Galactosylceramide + N-Acetylneuraminic acid unknown GM4 + Water |
details |
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| Gene Name: |
NEU2 |
| Uniprot ID: |
Q9Y3R4 |
| Protein Sequence: |
FASTA |
| Gene Sequence: |
FASTA |
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| Name: |
Sialidase-4
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| Reactions: |
| Reaction |
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| Galactosylceramide + N-Acetylneuraminic acid unknown GM4 + Water |
details |
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| Gene Name: |
NEU4 |
| Uniprot ID: |
Q8WWR8 |
| Protein Sequence: |
FASTA |
| Gene Sequence: |
FASTA |
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| Name: |
Lactase-phlorizin hydrolase
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| Reactions: |
| Reaction |
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| beta-Lactose + Water unknown Beta-D-Galactose + D-Glucose |
details |
| Glycosyl-N-acylsphingosine + Water unknown N-acylsphingosine + a sugar |
details |
| beta-Lactose + Water unknown Alpha-D-Glucose + Beta-D-Galactose |
details |
| Lactose + Water unknown D-Glucose + Beta-D-Galactose |
details |
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| Gene Name: |
LCT |
| Uniprot ID: |
P09848 |
| Protein Sequence: |
FASTA |
| Gene Sequence: |
FASTA |
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| Name: |
Lactosylceramide alpha-2,3-sialyltransferase
|
| Reactions: |
| Reaction |
|
| Cytidine monophosphate N-acetylneuraminic acid + beta-D-galactosyl-(1->4)-beta-D-glucosyl-(1<->1)-ceramide unknown Cytidine monophosphate + alpha-N-acetylneuraminyl-(2->3)-beta-D-galactosyl-(1->4)-beta-D-glucosyl-(1<->1)-ceramide |
details |
| CMP-N-acetylneuraminate + Lactosylceramide unknown CMP + GM3 |
details |
|
| Gene Name: |
ST3GAL5 |
| Uniprot ID: |
Q9UNP4 |
| Protein Sequence: |
FASTA |
| Gene Sequence: |
FASTA |
|
| Name: |
Beta-galactosidase
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| Reactions: |
| Reaction |
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| beta-Lactose + Water unknown Alpha-D-Glucose + Beta-D-Galactose |
details |
| beta-D-Galactosyl-(1->4)-beta-D-glucosyl-(1<->1)-ceramide + Water unknown beta-D-Glucosyl-(1<->1)-ceramide + Beta-D-Galactose |
details |
| D-Galactosyl-N-acetyl-D-galactosaminyl-(N-acetylneuraminyl)-D-galactosyl-D-glucosylceramide + Water unknown N-Acetyl-D-galactosaminyl-(N-acetylneuraminyl)-D-galactosyl-D-glucosylceramide + Beta-D-Galactose |
details |
| GM1 + Water unknown GM2 + Beta-D-Galactose |
details |
| + Water unknown + Beta-D-Galactose |
details |
|
| Gene Name: |
GLB1 |
| Uniprot ID: |
P16278 |
| Protein Sequence: |
FASTA |
| Gene Sequence: |
FASTA |
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| Name: |
Beta-1,4 N-acetylgalactosaminyltransferase 1
|
| Reactions: |
| Reaction |
|
| UDP-N-acetyl-alpha-D-galactosamine + O-(N-acetyl-alpha-neuraminyl)-(2->3)-O-beta-D-galactopyranosyl-(1->4)-beta-D-glucopyranosyl-(1<->1)-ceramide unknown Uridine 5'-diphosphate + O-2-(acetylamino)-2-deoxy-beta-D-galactopyranosyl-(1->4)-O-(N-acetyl-alpha-neuraminyl-(2->3))-O-beta-D-galactopyranosyl-(1->4)-beta-D-glucopyranosyl-(1<->1)-ceramide |
details |
| UDP-N-acetyl-D-galactosamine + Lactosylceramide unknown UDP + GA2 |
details |
| UDP-N-acetyl-D-galactosamine + GM3 unknown UDP + GM2 |
details |
| UDP-N-acetyl-D-galactosamine + GD3 unknown UDP + GD2 |
details |
| UDP-N-acetyl-D-galactosamine + GT3 unknown UDP + GT2 |
details |
|
| Gene Name: |
B4GALNT1 |
| Uniprot ID: |
Q00973 |
| Protein Sequence: |
FASTA |
| Gene Sequence: |
FASTA |
|
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| Name: |
Beta-1,3-galactosyltransferase 4
|
| Reactions: |
| Reaction |
|
| Uridine diphosphategalactose + an N-acetyl-beta-D-galactosaminyl-(1->4)-(alpha-N-acetylneuraminyl-(2->3))-beta-D-galactosyl-(1->4)-beta-D-glucosyl-(1<->1)-ceramide unknown Uridine 5'-diphosphate + a beta-D-galactosyl-(1->3)-N-acetyl-beta-D-galactosaminyl-(1->4)-(alpha-N-acetylneuraminyl-(2->3))-beta-D-galactosyl-(1->4)-beta-D-glucosyl-(1<->1)-ceramide |
details |
| UDP-D-galactose + GM2 unknown UDP + GM1 |
details |
| UDP-D-galactose + GD2 unknown UDP + GD1b |
details |
| UDP-D-galactose + GT2 unknown UDP + GT1c |
details |
| UDP-D-galactose + GA2 unknown UDP + GA1 |
details |
|
| Gene Name: |
B3GALT4 |
| Uniprot ID: |
O96024 |
| Protein Sequence: |
FASTA |
| Gene Sequence: |
FASTA |
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| Name: |
Lactosylceramide 1,3-N-acetyl-beta-D-glucosaminyltransferase
|
| Reactions: |
| Reaction |
|
| Uridine diphosphate-N-acetylglucosamine + beta-D-galactosyl-(1->4)-beta-D-glucosyl-(1<->1)-ceramide unknown Uridine 5'-diphosphate + N-acetyl-beta-D-glucosaminyl-(1->3)-beta-D-galactosyl-(1->4)-beta-D-glucosyl-(1<->1)-ceramide |
details |
| UDP-N-acetyl-D-glucosamine + Lactosylceramide unknown UDP + Lc3Cer |
details |
|
| Gene Name: |
B3GNT5 |
| Uniprot ID: |
Q9BYG0 |
| Protein Sequence: |
FASTA |
| Gene Sequence: |
FASTA |
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