| Record Information |
| Version |
3.5 |
| Creation Date |
2005-11-16 08:48:42 -0700 |
| Update Date |
2013-02-08 17:13:26 -0700 |
| HMDB ID |
HMDB04855 |
| Secondary Accession Numbers |
None |
| Metabolite Identification |
| Common Name |
Ganglioside GM1 (18:1/16:0) |
| Description |
Ganglioside GM1 (18:1/16:0) 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 GM1 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. 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 |
- 1-O-[O-(N-Acetyl-a-neuraminosyl)-(2)-O-[O-b-D-galactopyranosyl-(1)-2-(acetylamino)-2-deoxy-b-D-galactopyranosyl-(1)]-O-b-D-galactopyranosyl-(1)-b-D-glucopyranosyl]-Ceramide
- 1-O-[O-(N-Acetyl-a-neuraminosyl)-(2->3)-O-[O-b-D galactopyranosyl-(1->3)-2-(acetylamino)-2-deoxy-b-D-galactopyranosyl-(1->4)]-O-b-D-galactopyranosyl-(1->4)-b-D-glucopyranosyl]-Ceramide
- 1-O-[O-(N-Acetyl-alpha-neuraminosyl)-(2)-O-[O-beta-delta-galactopyranosyl-(1)-2-(acetylamino)-2-deoxy-beta-delta-galactopyranosyl-(1)]-O-beta-delta-galactopyranosyl-(1)-beta-delta-glucopyranosyl]-Ceramide
- 1-O-[O-(N-Acetyl-alpha-neuraminosyl)-(2->3)-O-[O-beta-D galactopyranosyl-(1->3)-2-(acetylamino)-2-deoxy-beta-delta-galactopyranosyl-(1->4)]-O-beta-delta-galactopyranosyl-(1->4)-beta-delta-glucopyranosyl]-Ceramide
- D-Galactosyl-1,3-beta-N-acetyl-D galactosaminyl-(N-acetylneuraminyl)-D-galactosyl-D-glucosyl-Nacylsphingosine
- D-Galactosyl-N-acetyl-D galactosaminyl-(N-acetylneuraminyl)-D-galactosyl-D-glucosylceramide
- delta-Galactosyl-1,3-beta-N-acetyl-D galactosaminyl-(N-acetylneuraminyl)-delta-galactosyl-delta-glucosyl-Nacylsphingosine
- delta-Galactosyl-N-acetyl-D galactosaminyl-(N-acetylneuraminyl)-delta-galactosyl-delta-glucosylceramide
- Gal-beta1->3GalNAc-beta1->4(Neu5Ac-alpha2->3)Gal-beta1->4Glc-beta1->1'Cer
- Gal-beta1->3GalNAc-beta1->4(Neu5Ac-alpha2>3)Gal-beta1->4Glc-beta1->1'Cer
- Ganglioside GM1
- GM1
- GM1a
- II3NeuAcGgOse4Cer
- N-Acetyl-Ganglioside GM1
- N-Acetylganglioside GM1
- NeuAc-GM1a
|
| Chemical Formula |
C71H127N3O31 |
| Average Molecular Weight |
1518.7696 |
| Monoisotopic Molecular Weight |
1517.845354361 |
| IUPAC Name |
(2S,4S,5R)-5-acetamido-2-{[(2R,3S,4R,5R,6S)-3-{[(2S,3R,4R,5R,6R)-3-acetamido-5-hydroxy-6-(hydroxymethyl)-4-{[(2R,3R,4S,5R,6R)-3,4,5-trihydroxy-6-(hydroxymethyl)oxan-2-yl]oxy}oxan-2-yl]oxy}-6-{[(2R,3S,4R,6R)-6-{[(2S,3R,4E)-2-hexadecanamido-3-hydroxyoctadec-4-en-1-yl]oxy}-4,5-dihydroxy-2-(hydroxymethyl)oxan-3-yl]oxy}-5-hydroxy-2-(hydroxymethyl)oxan-4-yl]oxy}-4-hydroxy-6-[(1S,2S)-1,2,3-trihydroxypropyl]oxane-2-carboxylic acid |
| Traditional IUPAC Name |
(2S,4S,5R)-5-acetamido-2-{[(2R,3S,4R,5R,6S)-3-{[(2S,3R,4R,5R,6R)-3-acetamido-5-hydroxy-6-(hydroxymethyl)-4-{[(2R,3R,4S,5R,6R)-3,4,5-trihydroxy-6-(hydroxymethyl)oxan-2-yl]oxy}oxan-2-yl]oxy}-6-{[(2R,3S,4R,6R)-6-{[(2S,3R,4E)-2-hexadecanamido-3-hydroxyoctadec |
| CAS Registry Number |
104443-62-1 |
| SMILES |
CCCCCCCCCCCCCCCC(=O)N[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]4O[C@H](CO)[C@H](O)[C@H](O)[C@H]4O)[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)C1O)[C@H](O)\C=C\CCCCCCCCCCCCC |
| InChI Identifier |
InChI=1S/C71H127N3O31/c1-5-7-9-11-13-15-17-19-21-23-25-27-29-31-43(82)42(74-50(85)32-30-28-26-24-22-20-18-16-14-12-10-8-6-2)39-96-67-59(92)57(90)61(48(37-78)99-67)101-69-60(93)65(105-71(70(94)95)33-44(83)51(72-40(3)80)64(104-71)53(86)45(84)34-75)62(49(38-79)100-69)102-66-52(73-41(4)81)63(55(88)47(36-77)97-66)103-68-58(91)56(89)54(87)46(35-76)98-68/h29,31,42-49,51-69,75-79,82-84,86-93H,5-28,30,32-39H2,1-4H3,(H,72,80)(H,73,81)(H,74,85)(H,94,95)/b31-29+/t42-,43+,44-,45-,46+,47+,48+,49+,51+,52+,53-,54-,55-,56-,57+,58+,59?,60+,61+,62-,63+,64?,65+,66-,67+,68-,69-,71-/m0/s1 |
| InChI Key |
BWZAKDPPVXHOTB-TWDSFERASA-N |
| Chemical Taxonomy |
| Kingdom |
Organic Compounds |
| Super Class |
Lipids |
| Class |
Sphingolipids |
| Sub Class |
Neutral Glycosphingolipids |
| Other Descriptors |
- Aliphatic Heteropolycyclic Compounds
- Gangliosides
- Hexose Oligosaccharides
|
| 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
- 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
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| Cellular locations |
|
| 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
|
| Biological Properties |
| Cellular Locations |
|
| Biofluid Locations |
Not Available
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| Tissue Location |
- Fibroblasts
- Brain
- Myelin
- Nerve Cells
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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 |
| Phenol Explorer Compound ID |
Not Available |
| Phenol Explorer Metabolite ID |
Not Available |
| FoodDB ID |
FDB023456 |
| KNApSAcK ID |
Not Available |
| Chemspider ID |
16744872  |
| KEGG Compound ID |
C04911  |
| BioCyc ID |
Not Available |
| BiGG ID |
Not Available |
| Wikipedia Link |
GM1  |
| NuGOwiki Link |
HMDB04855  |
| Metagene Link |
HMDB04855  |
| METLIN ID |
Not Available |
| PubChem Compound |
20057296  |
| PDB ID |
Not Available |
| ChEBI ID |
Not Available |
| References |
| Synthesis Reference |
Mikhalyov, I. I.; Molotkovsky, Jul. G. Synthesis and characteristics of fluorescent BODIPY-labeled gangliosides. Russian Journal of Bioorganic Chemistry (Translation of Bioorganicheskaya Khimiya) (2003), 29(2), 168-174. |
| Material Safety Data Sheet (MSDS) |
Not Available
|
| General References |
- Rost KL, Brockmoller J, Weber W, Roots I: Multiple-dose pharmacokinetics of ganglioside GM1 after intravenous and intramuscular administration to healthy volunteers. Clin Pharmacol Ther. 1991 Aug;50(2):141-9.
Pubmed: 1868675
- Sadeghlar F, Remmel N, Breiden B, Klingenstein R, Schwarzmann G, Sandhoff K: Physiological relevance of sphingolipid activator proteins in cultured human fibroblasts. Biochimie. 2003 Mar-Apr;85(3-4):439-48.
Pubmed: 12770782
- Vogel A, Furst W, Abo-Hashish MA, Lee-Vaupel M, Conzelmann E, Sandhoff K: Identity of the activator proteins for the enzymatic hydrolysis of sulfatide, ganglioside GM1, and globotriaosylceramide. Arch Biochem Biophys. 1987 Dec;259(2):627-38.
Pubmed: 3426245
- Gregson NA, Koblar S, Hughes RA: Antibodies to gangliosides in Guillain-Barre syndrome: specificity and relationship to clinical features. Q J Med. 1993 Feb;86(2):111-7.
Pubmed: 8464986
- Avrova NF, Zakharova IO, Tyurin VA, Tyurina YY, Gamaley IA, Schepetkin IA: Different metabolic effects of ganglioside GM1 in brain synaptosomes and phagocytic cells. Neurochem Res. 2002 Aug;27(7-8):751-9.
Pubmed: 12374210
- Shoemaker JD, Elliott WH: Automated screening of urine samples for carbohydrates, organic and amino acids after treatment with urease. J Chromatogr. 1991 Jan 2;562(1-2):125-38.
Pubmed: 2026685
- Takaura N, Yagi T, Maeda M, Nanba E, Oshima A, Suzuki Y, Yamano T, Tanaka A: Attenuation of ganglioside GM1 accumulation in the brain of GM1 gangliosidosis mice by neonatal intravenous gene transfer. Gene Ther. 2003 Aug;10(17):1487-93.
Pubmed: 12900764
- Cacic M: Gangliosides of anencephalic and fetal brain--immunostaining on thin-layer chromatograms. Neuroreport. 1995 Jan 26;6(2):389-93.
Pubmed: 7538811
- Mellgren SI: [Peripheral neuropathies] Tidsskr Nor Laegeforen. 1995 May 30;115(14):1735-8.
Pubmed: 7785034
- Blennow K, Wallin A: Clinical heterogeneity of probable Alzheimer's disease. J Geriatr Psychiatry Neurol. 1992 Apr-Jun;5(2):106-13.
Pubmed: 1590911
- Matsuda J, Suzuki O, Oshima A, Ogura A, Noguchi Y, Yamamoto Y, Asano T, Takimoto K, Sukegawa K, Suzuki Y, Naiki M: Beta-galactosidase-deficient mouse as an animal model for GM1-gangliosidosis. Glycoconj J. 1997 Sep;14(6):729-36.
Pubmed: 9337086
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| Enzymes |
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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: |
Sialidase-3
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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: |
NEU3 |
| Uniprot ID: |
Q9UQ49  |
| 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: |
Lactosylceramide alpha-2,3-sialyltransferase
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| Reactions: |
- CMP-N-acetylneuraminate + beta-D-galactosyl-(1->4)-beta-D-glucosyl-(11)-ceramide = CMP + alpha-N-acetylneuraminyl-(2->3)-beta-D-galactosyl-(1->4)-beta-D- glucosyl-(11)-ceramide [RN:R03488 R05937]
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| Gene Name: |
ST3GAL5 |
| Uniprot ID: |
Q9UNP4  |
| Protein Sequence: |
FASTA |
| Gene Sequence: |
FASTA |
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| Name: |
Beta-galactosidase
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| Reactions: |
- Hydrolysis of terminal non-reducing beta-D-galactose residues in beta-D-galactosides ALL_REAC (other) R01100 R01105 R01678 R03355 R04633 R04783 R05112 R05994(G) R06010(G) R06098(G) R06099(G) R06114(G) R06144(G) R06202(G) R07807(G)
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| Gene Name: |
GLB1 |
| Uniprot ID: |
P16278  |
| Protein Sequence: |
FASTA |
| Gene Sequence: |
FASTA |
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| Name: |
Beta-1,3-galactosyltransferase 4
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| Reactions: |
- UDP-galactose + N-acetyl-D-galactosaminyl-(N-acetylneuraminyl)-D-galactosyl-(1->4)- beta-D-glucosyl-N-acylsphingosine = UDP + D-galactosyl-(1->3)-beta-N-acetyl-D-galactosaminyl-(N- acetylneuraminyl)-D-galactosyl-D-glucosyl-N-acylsphingosine [RN:R04631 R05941]
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| Gene Name: |
B3GALT4 |
| Uniprot ID: |
O96024  |
| Protein Sequence: |
FASTA |
| Gene Sequence: |
FASTA |
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