Human Metabolome Database Version 3.5

Showing metabocard for Ganglioside GM2 (d18:0/12:0) (HMDB11894)

Record Information
Version 3.5
Creation Date 2009-03-24 10:18:48 -0600
Update Date 2013-07-24 12:08:52 -0600
HMDB ID HMDB11894
Secondary Accession Numbers None
Metabolite Identification
Common Name Ganglioside GM2 (d18:0/12:0)
Description Ganglioside GM2 (d18:0/12:0) is a ganglioside. A ganglioside is a compound composed of a glycosphingolipid (ceramide and oligosaccharide) with one or more sialic acids (AKA n-acetylneuraminic acid, NANA) linked on the sugar chain. The 60+ known gangliosides differ mainly in the position and number of NANA residues. It is a component of the cell plasma membrane that modulates cell signal transduction events. It appears that they concentrate in lipid rafts. They have recently been found to be highly important in immunology. Natural and semisynthetic gangliosides are considered possible therapeutics for neurodegenerative disorders. Gangliosides are more complex glycosphingolipids in which oligosaccharide chains containing N-acetylneuraminic acid (NeuNAc) are attached to a ceramide. NeuNAc, an acetylated derivative of the carbohydrate sialic acid, makes the head groups of Gangliosides anionic. NB: the M in GM2 stands for monosialo, i.e., one NeuNAc residue. GM2 is the second monosialo ganglioside characterized, thus the subscript 2. Their structural diversity results from variation in the composition and sequence of the sugar residues. In all Gangliosides, the ceramide is linked through its C-1 to a beta-glucosyl residue, which, in turn, is bound to a beta-galactosyl residue. (Wikipedia) Particularly, Ganglioside GM2 (d18:0/12:0) is a GM2 ganglioside. 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 Thumb
Download: MOL | SDF | PDB | SMILES | InChI
Display: 2D Structure | 3D Structure
Synonyms Not Available
Chemical Formula C62H112N2O26
Average Molecular Weight 1301.5505
Monoisotopic Molecular Weight 1300.750331766
IUPAC Name (2S,4S,5R)-2-{[(2R,3S,4R,5R,6S)-3-{[(2S,3R,4R,5R,6R)-4,5-dihydroxy-6-(hydroxymethyl)-3-(2-oxopropyl)oxan-2-yl]oxy}-6-{[(2R,3S,4R,5R,6R)-6-{[(2S,3R)-2-dodecanamido-3-hydroxyoctadecyl]oxy}-4,5-dihydroxy-2-(hydroxymethyl)oxan-3-yl]oxy}-5-hydroxy-2-(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-{[(2R,3S,4R,5R,6S)-3-{[(2S,3R,4R,5R,6R)-4,5-dihydroxy-6-(hydroxymethyl)-3-(2-oxopropyl)oxan-2-yl]oxy}-6-{[(2R,3S,4R,5R,6R)-6-{[(2S,3R)-2-dodecanamido-3-hydroxyoctadecyl]oxy}-4,5-dihydroxy-2-(hydroxymethyl)oxan-3-yl]oxy}-5-hydroxy-2-(hydroxyme
CAS Registry Number Not Available
SMILES CCCCCCCCCCCCCCC[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]3CC(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)CCCCCCCCCCC
InChI Identifier InChI=1S/C62H112N2O26/c1-5-7-9-11-13-15-16-17-18-20-21-23-25-27-40(71)39(64-46(74)28-26-24-22-19-14-12-10-8-6-2)35-83-59-52(79)51(78)54(44(33-67)85-59)87-60-53(80)57(55(45(34-68)86-60)88-58-38(29-36(3)69)48(75)50(77)43(32-66)84-58)90-62(61(81)82)30-41(72)47(63-37(4)70)56(89-62)49(76)42(73)31-65/h38-45,47-60,65-68,71-73,75-80H,5-35H2,1-4H3,(H,63,70)(H,64,74)(H,81,82)/t38-,39+,40-,41+,42-,43-,44-,45-,47-,48-,49-,50+,51-,52-,53-,54-,55+,56?,57-,58+,59-,60+,62+/m1/s1
InChI Key UKUHZABZYBYURP-UEJXXIOUSA-N
Chemical Taxonomy
Kingdom Organic Compounds
Super Class Carbohydrates and Carbohydrate Conjugates
Class Tetrasaccharides
Sub Class Tetrahexoses
Other Descriptors
  • Aliphatic Heteropolycyclic Compounds
Substituents
  • 1,2 Diol
  • 1,3 Aminoalcohol
  • Acetal
  • Alkyl Glycoside
  • Carboxamide Group
  • Carboxylic Acid
  • Ceramide
  • Fatty Acyl Glycoside
  • Glyco Amino Acid
  • Glycosyl Compound
  • Ketone
  • N Acetylneuraminic Acid
  • N Acyl Amine
  • Neuraminic Acid
  • O Glycosyl Compound
  • Oxane
  • Primary Alcohol
  • Pyranoid Amino Acid
  • Secondary Alcohol
  • Secondary Carboxylic Acid Amide
  • Simple Glycosylsphingosine
  • Sugar Acid
Direct Parent Tetrahexoses
Ontology
Status Expected and Not Quantified
Origin
  • Endogenous
Biofunction
  • Cell signaling
  • Energy source
  • Membrane component
Application
  • Surfactants and emulsifiers
Cellular locations
  • Membrane
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 0.21 g/L ALOGPS
LogP 1.93 ALOGPS
LogP 2.31 ChemAxon
LogS -3.80 ALOGPS
pKa (strongest acidic) 2.82 ChemAxon
pKa (strongest basic) -3.5 ChemAxon
Hydrogen Acceptor Count 26 ChemAxon
Hydrogen Donor Count 16 ChemAxon
Polar Surface Area 449.4 A2 ChemAxon
Rotatable Bond Count 45 ChemAxon
Refractivity 316.85 ChemAxon
Polarizability 143.77 ChemAxon
Formal Charge 0 ChemAxon
Physiological Charge -1 ChemAxon
Spectra
Not Available
Biological Properties
Cellular Locations
  • Membrane
Biofluid Locations Not Available
Tissue Location Not Available
Pathways Not Available
Normal Concentrations
Not Available
Abnormal Concentrations
Not Available
Associated Disorders and Diseases
Disease References None
Associated OMIM IDs None
DrugBank ID Not Available
DrugBank Metabolite ID Not Available
Phenol Explorer Compound ID Not Available
Phenol Explorer Metabolite ID Not Available
FoodDB ID FDB028563
KNApSAcK ID Not Available
Chemspider ID Not Available
KEGG Compound ID Not Available
BioCyc ID Not Available
BiGG ID Not Available
Wikipedia Link Not Available
NuGOwiki Link HMDB11894 Link_out
Metagene Link HMDB11894 Link_out
METLIN ID Not Available
PubChem Compound 53481170 Link_out
PDB ID Not Available
ChEBI ID Not Available
References
Synthesis Reference Not Available
Material Safety Data Sheet (MSDS) Not Available
General References Not Available

Enzymes
Name: Alpha-N-acetylneuraminide alpha-2,8-sialyltransferase
Reactions: Not Available
Gene Name: ST8SIA1
Uniprot ID: Q92185 Link_out
Protein Sequence: FASTA
Gene Sequence: FASTA
Name: Sialidase-3
Reactions: Not Available
Gene Name: NEU3
Uniprot ID: Q9UQ49 Link_out
Protein Sequence: FASTA
Gene Sequence: FASTA
Name: Globoside alpha-1,3-N-acetylgalactosaminyltransferase 1
Reactions: Not Available
Gene Name: GBGT1
Uniprot ID: Q8N5D6 Link_out
Protein Sequence: FASTA
Gene Sequence: FASTA
Name: N-acetylglucosaminyl-phosphatidylinositol de-N-acetylase
Reactions: Not Available
Gene Name: PIGL
Uniprot ID: Q9Y2B2 Link_out
Protein Sequence: FASTA
Gene Sequence: FASTA
Name: Lactosylceramide alpha-2,3-sialyltransferase
Reactions: Not Available
Gene Name: ST3GAL5
Uniprot ID: Q9UNP4 Link_out
Protein Sequence: FASTA
Gene Sequence: FASTA
Name: Beta-1,4 N-acetylgalactosaminyltransferase 1
Reactions: Not Available
Gene Name: B4GALNT1
Uniprot ID: Q00973 Link_out
Protein Sequence: FASTA
Gene Sequence: FASTA
Name: Phosphatidylinositol N-acetylglucosaminyltransferase subunit Q
Reactions: Not Available
Gene Name: PIGQ
Uniprot ID: Q9BRB3 Link_out
Protein Sequence: FASTA
Gene Sequence: FASTA
Name: Phosphatidylinositol N-acetylglucosaminyltransferase subunit A
Reactions: Not Available
Gene Name: PIGA
Uniprot ID: P37287 Link_out
Protein Sequence: FASTA
Gene Sequence: FASTA
Name: Phosphatidylinositol N-acetylglucosaminyltransferase subunit H
Reactions: Not Available
Gene Name: PIGH
Uniprot ID: Q14442 Link_out
Protein Sequence: FASTA
Gene Sequence: FASTA
Name: Phosphatidylinositol N-acetylglucosaminyltransferase subunit P
Reactions: Not Available
Gene Name: PIGP
Uniprot ID: P57054 Link_out
Protein Sequence: FASTA
Gene Sequence: FASTA
Name: Phosphatidylinositol N-acetylglucosaminyltransferase subunit C
Reactions: Not Available
Gene Name: PIGC
Uniprot ID: Q92535 Link_out
Protein Sequence: FASTA
Gene Sequence: FASTA
Name: Beta-1,3-galactosyltransferase 4
Reactions: Not Available
Gene Name: B3GALT4
Uniprot ID: O96024 Link_out
Protein Sequence: FASTA
Gene Sequence: FASTA
Name: Ganglioside GM2 activator
Reactions: Not Available
Gene Name: GM2A
Uniprot ID: P17900 Link_out
Protein Sequence: FASTA
Gene Sequence: FASTA
Name: T-cell surface glycoprotein CD1e, membrane-associated
Reactions: Not Available
Gene Name: CD1E
Uniprot ID: P15812 Link_out
Protein Sequence: FASTA
Gene Sequence: FASTA
Name: Epididymal secretory protein E1
Reactions: Not Available
Gene Name: NPC2
Uniprot ID: P61916 Link_out
Protein Sequence: FASTA
Gene Sequence: FASTA
Name: Antigen-presenting glycoprotein CD1d
Reactions: Not Available
Gene Name: CD1D
Uniprot ID: P15813 Link_out
Protein Sequence: FASTA
Gene Sequence: FASTA
Name: GPI mannosyltransferase 1
Reactions: Not Available
Gene Name: PIGM
Uniprot ID: Q9H3S5 Link_out
Protein Sequence: FASTA
Gene Sequence: FASTA
Name: Phosphatidylinositol-glycan biosynthesis class W protein
Reactions: Not Available
Gene Name: PIGW
Uniprot ID: Q7Z7B1 Link_out
Protein Sequence: FASTA
Gene Sequence: FASTA
Name: Phosphatidylinositol-glycan biosynthesis class X protein
Reactions: Not Available
Gene Name: PIGX
Uniprot ID: Q8TBF5 Link_out
Protein Sequence: FASTA
Gene Sequence: FASTA
Name: GPI mannosyltransferase 4
Reactions: Not Available
Gene Name: PIGZ
Uniprot ID: Q86VD9 Link_out
Protein Sequence: FASTA
Gene Sequence: FASTA
Name: Lactosylceramide 1,3-N-acetyl-beta-D-glucosaminyltransferase
Reactions: Not Available
Gene Name: B3GNT5
Uniprot ID: Q9BYG0 Link_out
Protein Sequence: FASTA
Gene Sequence: FASTA
Name: Beta-1,3-galactosyltransferase 5
Reactions: Not Available
Gene Name: B3GALT5
Uniprot ID: Q9Y2C3 Link_out
Protein Sequence: FASTA
Gene Sequence: FASTA
Name: GPI mannosyltransferase 3
Reactions: Not Available
Gene Name: PIGB
Uniprot ID: Q92521 Link_out
Protein Sequence: FASTA
Gene Sequence: FASTA
Name: Phosphatidylinositol-glycan biosynthesis class F protein
Reactions: Not Available
Gene Name: PIGF
Uniprot ID: Q07326 Link_out
Protein Sequence: FASTA
Gene Sequence: FASTA
Name: GPI ethanolamine phosphate transferase 2
Reactions: Not Available
Gene Name: PIGG
Uniprot ID: Q5H8A4 Link_out
Protein Sequence: FASTA
Gene Sequence: FASTA
Name: GPI ethanolamine phosphate transferase 1
Reactions: Not Available
Gene Name: PIGN
Uniprot ID: O95427 Link_out
Protein Sequence: FASTA
Gene Sequence: FASTA
Name: GPI ethanolamine phosphate transferase 3
Reactions: Not Available
Gene Name: PIGO
Uniprot ID: Q8TEQ8 Link_out
Protein Sequence: FASTA
Gene Sequence: FASTA
Name: GPI transamidase component PIG-S
Reactions: Not Available
Gene Name: PIGS
Uniprot ID: Q96S52 Link_out
Protein Sequence: FASTA
Gene Sequence: FASTA
Name: GPI transamidase component PIG-T
Reactions: Not Available
Gene Name: PIGT
Uniprot ID: Q969N2 Link_out
Protein Sequence: FASTA
Gene Sequence: FASTA
Name: Phosphatidylinositol glycan anchor biosynthesis class U protein
Reactions: Not Available
Gene Name: PIGU
Uniprot ID: Q9H490 Link_out
Protein Sequence: FASTA
Gene Sequence: FASTA
Name: GPI mannosyltransferase 2
Reactions: Not Available
Gene Name: PIGV
Uniprot ID: Q9NUD9 Link_out
Protein Sequence: FASTA
Gene Sequence: FASTA
Name: Phosphatidylinositol N-acetylglucosaminyltransferase subunit Y
Reactions: Not Available
Gene Name: PIGY
Uniprot ID: Q3MUY2 Link_out
Protein Sequence: FASTA
Gene Sequence: FASTA
Name: Pleckstrin homology domain-containing family A member 8
Reactions: Not Available
Gene Name: PLEKHA8
Uniprot ID: Q96JA3 Link_out
Protein Sequence: FASTA
Gene Sequence: FASTA
Name: Non-lysosomal glucosylceramidase
Reactions: Not Available
Gene Name: GBA2
Uniprot ID: Q9HCG7 Link_out
Protein Sequence: FASTA
Gene Sequence: FASTA
Name: GPI-anchor transamidase
Reactions: Not Available
Gene Name: PIGK
Uniprot ID: Q92643 Link_out
Protein Sequence: FASTA
Gene Sequence: FASTA
Name: Glycosylphosphatidylinositol anchor attachment 1 protein
Reactions: Not Available
Gene Name: GPAA1
Uniprot ID: O43292 Link_out
Protein Sequence: FASTA
Gene Sequence: FASTA
Name: Protein PLEKHA9
Reactions: Not Available
Gene Name: PLEKHA9
Uniprot ID: O95397 Link_out
Protein Sequence: FASTA
Gene Sequence: FASTA
Name: Glycolipid transfer protein domain-containing protein 2
Reactions: Not Available
Gene Name: GLTPD2
Uniprot ID: A6NH11 Link_out
Protein Sequence: FASTA
Gene Sequence: FASTA
Name: Putative uncharacterized protein PLEKHA8
Reactions: Not Available
Gene Name: PLEKHA8
Uniprot ID: B5MDU3 Link_out
Protein Sequence: FASTA
Gene Sequence: FASTA
Name: T-cell surface glycoprotein CD1a
Reactions: Not Available
Gene Name: CD1A
Uniprot ID: P06126 Link_out
Protein Sequence: FASTA
Gene Sequence: FASTA
Name: Glycolipid transfer protein domain-containing protein 1
Reactions: Not Available
Gene Name: GLTPD1
Uniprot ID: Q5TA50 Link_out
Protein Sequence: FASTA
Gene Sequence: FASTA
Name: T-cell surface glycoprotein CD1c
Reactions: Not Available
Gene Name: CD1C
Uniprot ID: P29017 Link_out
Protein Sequence: FASTA
Gene Sequence: FASTA
Name: Lipopolysaccharide-binding protein
Reactions: Not Available
Gene Name: LBP
Uniprot ID: P18428 Link_out
Protein Sequence: FASTA
Gene Sequence: FASTA
Name: Putative uncharacterized protein DKFZp434L0435
Reactions: Not Available
Gene Name: DKFZp434L0435
Uniprot ID: Q9UFH6 Link_out
Protein Sequence: FASTA
Gene Sequence: FASTA
Name: T-cell surface glycoprotein CD1b
Reactions: Not Available
Gene Name: CD1B
Uniprot ID: P29016 Link_out
Protein Sequence: FASTA
Gene Sequence: FASTA
Name: Glycolipid transfer protein
Reactions: Not Available
Gene Name: GLTP
Uniprot ID: Q9NZD2 Link_out
Protein Sequence: FASTA
Gene Sequence: FASTA