These functions are mainly governed by the glycan moiety, and changes in the structures of gangliosides occur under pathological conditions, particularly in neuro-ectoderm-derived cancers. The GM2 ganglioside is composed of N-Acetyl-D-galactose-beta-1,4-[N-Acetylneuraminidate- alpha-2,3-]-Galactose-beta-1,4-glucose-alpha-ceramide. (eds. Crystallographic structure of human beta-hexosaminidase A: interpretation of Tay-Sachs mutations and loss of GM2 ganglioside hydrolysis. 12-17 Human Cancer Ganglioside GM2 on the K562 cell line is recognized as a target structure by human natural killer cells Iwao Ando Division of Surgical Oncology, John Wayne Cancer Clinic, Jonsson Comprehensive Cancer Center, UCLA School of Medicine, Los Angeles, CA 90024 Search for more papers by this author Dave S. B. Hoon Shown in both of these figures is the stearoyl fatty acid form of ceramide. Applications Products Services Support. They are essential compounds at the outer leaflet of the plasma membrane, where they interact with phospholipids, cholesterol, and transmembrane proteins, forming lipid rafts. Ganglioside GM2 is degraded in the lysosome by -hexosaminidase A. As of 2004, 188 gangliosides have been . Buildup of the lipid causes nerve cells to become swollen, leading . ), Structure and Function of Gangliosides, New York, Plenum, 1980. N -acetylneuraminic acid, NANA) linked on the sugar chain. They contain a sialated polysaccharide chain linked to ceramide through a -glycosidic linkage. -hexosaminidase has two major isozymes, hexosaminidase a (hex a), which is composed of alpha and beta subunits, and hexosaminidase b (hex b), Ganglioside GM2 | C67H121N3O26 | CID 9898635 - structure, chemical names, physical and chemical properties, classification, patents, literature, biological activities . Abstract Although human NK cells lyse a wide spectrum of target cells, the precise target structure recognized by NK cells has not yet been elucidated. Europe PMC. English: Structure of GM1, GM2, GM3, referenced from Principles of Biochemistry, 3rd Edition, by Donald Voet, Judith Voet and Charlotte Pratt, p.253 Date 26 August 2012 a Structure of GM1 ganglioside.b Density map of glycosidic linkage torsion angles of the experimentally validated MD trajectory of the GM1, GM2, and GM3 oligosaccharides. G refers to ganglioside, the M is for monosialic (as in it has one sialic acid ), and 2 refers to the fact that it was the second monosialic ganglioside discovered. A perspective structure of GM2 ganglioside is shown in figure 3 and the corresponding ball-and-stick model is shown in figure 4. Beta-hexosaminidase A and the ganglioside GM2 activator protein work . It is associated with GM2 gangliosidoses such as Tay-Sachs disease. (2000) determined the crystal structure of GM2 activator at 2.0-angstrom resolution, which revealed a previously unobserved fold whose main feature is an 8-stranded cup-shaped antiparallel beta-pleated . Advanced Search. The flexible recognition of these glycan receptors by MuV may explain the unique tropism and . Table 1. [1] Contents 1 See also 2 Additional images The antigen-binding groove houses four interlinked hydrophobic channels that are occupied by the alkyl chains of the glycolipid plus two detergent molecules. In addition, GM2A levels are elevated in AD brain tissue in our cohort and are elevated in AD cerebrospinal fluid (CSF) in a cohort of 384 individuals (202 control vs. 182 AD). In 1962 Svenner- holm identified the major neuronal storage compound in Tay-Sachs disease as ganglioside GM2. Tay-Sachs disease is caused by a genetic mutation that impairs proper degradation of gangliosides. 3D GM2 ganglioside Molecular Formula CHNO Average mass 1398.709 Da Monoisotopic mass 1397.839478 Da ChemSpider ID 26286730 - Double-bond stereo - 23 of 23 defined stereocentres More details: Names Properties Searches Spectra Vendors Articles More Names and Synonyms Validated by Experts, Validated by Users, Non-Validated, Removed by Users Ganglioside GM2 (d18:0/25:0) | C75H138N2O26 | CID 53481182 - structure, chemical names, physical and chemical properties, classification, patents, literature . Here, using a large-scale glycan array and X-ray crystallography, we show that MuV recognizes sialyl Lewis X and GM2 ganglioside as receptors, in addition to a previously reported MuV receptor, a trisaccharide containing an 2,3-linked sialic acid. DOI: 10.1016/j.jmb.2006.04.004 Abstract Lysosomal beta-hexosaminidase A (Hex A) is essential for the degradation of GM2 gangliosides in the central and peripheral nervous system. A ganglioside is a molecule composed of a glycosphingolipid ( ceramide and oligosaccharide) with one or more sialic acids (e.g. Product Category. This enzyme removes the terminal N -acetylgalactosaminyl residue. US EN. The enzyme requires the presence of an additional protein termed 'GM2 activator' encoded by the GM2A gene. Volume 40, Issue 1p. We present here the crystal structures of CD1b in complex with either phosphatidylinositol or ganglioside GM2 at 2.3 A and 2.8 A resolutions, respectively. Series Basic structure Abbreviation; Globo: GalNAc1 3 Gal1 4Gal1 4Glc1 1 'Cer . The GM2A gene provides instructions for making a protein called the ganglioside GM2 activator. -Hexosaminidase A is a heterodimer consisting of an - and -subunit. Search Within. (GalNAcT or GA2/GM2/GD2/GT2 synthase), UDP-Gal: GA2/GM2 . About . https://orcid.org. GM2 (ganglioside) In organic chemistry, GM2 is a type of ganglioside. Gangliosides are acidic glycosphingolipids containing one or more sialic acid residues. Find ganglioside gm2 and related products for scientific research at MilliporeSigma. Carbohydrate structures of glycosphingolipids. Gangliosides are major constituents of neuronal cell membranes and endoplasmic reticulum. The abundance of ganglioside GM2 (structure 6, P = 0.042) was significantly elevated in serum samples from patients with cholangiocarcinoma, compared with serum samples from healthy controls. The glycosidic torsions of the carbohydrate moieties of GM1 in DMSO calculated by NOE-based molecular mechanics [] (), the carbohydrate moieties of GM1 in aqueous solution calculated by NOE-based molecular modeling [] (), and . Structure Search. GM2 gangliosidosis are a group of pathologies characterized by GM2 ganglioside accumulation into the lysosome due to mutations on the genes encoding for the -hexosaminidases subunits or the. This protein is necessary for the normal function of an enzyme called beta-hexosaminidase A, which plays a critical role in the brain and spinal cord (central nervous system). Its structure elu- cidation by Makita and Yamakawa followed shortly after this. gm2 gangliosidoses are a group of recessively inherited disorders characterized by the accumulation of the gm2 ganglioside within neuronal cells secondary to a deficiency in -hexosaminidase activity. With the progress in mass spectrometry analysis of gangliosides, their role in cancer progression can be now investigated in more detail. Structure, properties, spectra, suppliers and links for: Ganglioside GM2 (d18:1/18:0), 104443-57-4. Menu. The genetic disorder Tay-Sachs disease, found mainly in Jewish populations, results from the harmful accumulation of gangliosides in the nerve cells of the brain and other tissues. Figure 1 depicts a common brain ganglioside, GM1. Sign in | Create an account. Structure of GM1 ganglioside. Figure 1. Products Building Blocks Explorer Technical Documents Site Content Papers Genes Chromatograms. Wright et al. The GM2 activator is a low molecular weight, soluble protein that binds to the GM2 ganglioside, extracts it from the membrane, . GM2A is a lipid transport protein that binds to and stimulates ganglioside GM2 degradation, and mutations in this gene result in GM2 gangliosidosis, AB variant [ 8 ]. In order to define a possible relationship be. For classification of gangliosides see Svennerholm, L., et al. NeuNAc, an acetylated derivative of the carbohydrate sialic acid, makes the head groups of gangliosides anionic at pH 7, which distinguishes them from globosides. Accumulation of GM2 leads to severely debilitating neurodegeneration associated with Tay-Sachs disease (TSD), Sandoff disease (SD) and AB variant.
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