Record Information
Version3.6
Creation Date2009-03-24 16:18:50 UTC
Update Date2013-07-24 18:08:52 UTC
HMDB IDHMDB11896
Secondary Accession NumbersNone
Metabolite Identification
Common NameGanglioside GM2 (d18:0/16:0)
DescriptionGanglioside GM2 (d18:0/16:0) 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 (d18:0/16:0) 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
SynonymsNot Available
Chemical FormulaC66H120N2O26
Average Molecular Weight1357.6568
Monoisotopic Molecular Weight1356.812932022
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-hexadecanamido-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-hexadecanamido-3-hydroxyoctadecyl]oxy}-4,5-dihydroxy-2-(hydroxymethyl)oxan-3-yl]oxy}-5-hydroxy-2-(hydroxy
CAS Registry NumberNot 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)CCCCCCCCCCCCCCC
InChI Identifier
InChI=1S/C66H120N2O26/c1-5-7-9-11-13-15-17-19-21-23-25-27-29-31-44(75)43(68-50(78)32-30-28-26-24-22-20-18-16-14-12-10-8-6-2)39-87-63-56(83)55(82)58(48(37-71)89-63)91-64-57(84)61(59(49(38-72)90-64)92-62-42(33-40(3)73)52(79)54(81)47(36-70)88-62)94-66(65(85)86)34-45(76)51(67-41(4)74)60(93-66)53(80)46(77)35-69/h42-49,51-64,69-72,75-77,79-84H,5-39H2,1-4H3,(H,67,74)(H,68,78)(H,85,86)/t42-,43+,44-,45+,46-,47-,48-,49-,51-,52-,53-,54+,55-,56-,57-,58-,59+,60?,61-,62+,63-,64+,66+/m1/s1
InChI KeyDHKHQJJJGFMATL-NIFOSJJTSA-N
Chemical Taxonomy
KingdomOrganic Compounds
Super ClassLipids
ClassSphingolipids
Sub ClassGangliosides
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
  • Hexose Tetrasaccharide
  • Ketone
  • N Acetylneuraminic Acid
  • N Acyl Amine
  • Neuraminic Acid
  • O Glycosyl Compound
  • Oxane
  • Primary Alcohol
  • Pyranoid Amino Acid
  • Saccharide
  • Secondary Alcohol
  • Secondary Carboxylic Acid Amide
  • Simple Glycosylsphingosine
  • Sugar Acid
Direct ParentGangliosides
Ontology
StatusExpected and Not Quantified
Origin
  • Endogenous
  • Food
Biofunction
  • Cell signaling
  • Energy source
  • Fuel and energy storage
  • Fuel or energy source
  • Membrane component
  • Membrane integrity/stability
Application
  • Nutrients
  • Stabilizers
  • Surfactants and Emulsifiers
Cellular locations
  • Extracellular
  • Membrane
Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting PointNot AvailableNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Predicted Properties
PropertyValueSource
water solubility0.1 g/LALOGPS
logP2.77ALOGPS
logP4.09ChemAxon
logS-4.1ALOGPS
pKa (strongest acidic)2.82ChemAxon
pKa (strongest basic)-3.5ChemAxon
physiological charge-1ChemAxon
hydrogen acceptor count26ChemAxon
hydrogen donor count16ChemAxon
polar surface area449.4ChemAxon
rotatable bond count49ChemAxon
refractivity335.25ChemAxon
polarizability152.28ChemAxon
Spectra
SpectraNot Available
Biological Properties
Cellular Locations
  • Extracellular
  • Membrane
Biofluid LocationsNot Available
Tissue LocationNot Available
PathwaysNot Available
Normal Concentrations
Not Available
Abnormal Concentrations
Not Available
Associated Disorders and Diseases
Disease ReferencesNone
Associated OMIM IDsNone
DrugBank IDNot Available
DrugBank Metabolite IDNot Available
Phenol Explorer Compound IDNot Available
Phenol Explorer Metabolite IDNot Available
FoodDB IDFDB028565
KNApSAcK IDNot Available
Chemspider IDNot Available
KEGG Compound IDNot Available
BioCyc IDNot Available
BiGG IDNot Available
Wikipedia LinkNot Available
NuGOwiki LinkHMDB11896
Metagene LinkHMDB11896
METLIN IDNot Available
PubChem Compound53481172
PDB IDNot Available
ChEBI IDNot Available
References
Synthesis ReferenceNot Available
Material Safety Data Sheet (MSDS)Not Available
General ReferencesNot Available

Enzymes

Gene Name:
NEU3
Uniprot ID:
Q9UQ49
Gene Name:
B3GALT4
Uniprot ID:
O96024
Gene Name:
GM2A
Uniprot ID:
P17900
Gene Name:
NPC2
Uniprot ID:
P61916
Gene Name:
CD1D
Uniprot ID:
P15813
Gene Name:
PIGM
Uniprot ID:
Q9H3S5
Gene Name:
PIGZ
Uniprot ID:
Q86VD9
Gene Name:
B3GALT5
Uniprot ID:
Q9Y2C3
Gene Name:
PIGB
Uniprot ID:
Q92521
Gene Name:
PIGS
Uniprot ID:
Q96S52
Gene Name:
PIGT
Uniprot ID:
Q969N2
Gene Name:
PIGV
Uniprot ID:
Q9NUD9
Gene Name:
GBA2
Uniprot ID:
Q9HCG7
Gene Name:
PIGK
Uniprot ID:
Q92643
Gene Name:
PLEKHA9
Uniprot ID:
O95397
Gene Name:
PLEKHA8
Uniprot ID:
B5MDU3
Gene Name:
CD1A
Uniprot ID:
P06126
Gene Name:
CD1C
Uniprot ID:
P29017
Gene Name:
LBP
Uniprot ID:
P18428
Gene Name:
DKFZp434L0435
Uniprot ID:
Q9UFH6
Gene Name:
CD1B
Uniprot ID:
P29016
Gene Name:
GLTP
Uniprot ID:
Q9NZD2