Disease relevance of Gangliotetraose

• Neuronal gangliosides (gangliotetraose series) were found in varying amounts in all medulloblastomas [1].
• Although cells are usually resistant to homologous complement, the IgM antibody against gangliotetraose (Gg4), an asialo-oligosaccharide of GM1, was found to cause cytolysis of HIV-1 infected cells by homologous complement [2].
• Differences in ganglioside patterns were observed, with neuroblastomas from patients who were either disease positive or dead of disease tending to have more monosialogangliosides and fewer gangliotetraose gangliosides of the B series (formula; see text) than tumors from patients who were disease-free [3].
• Low levels of CSF gangliotetraose-series gangliosides in West syndrome: implication of brain maturation disturbance [4].

High impact information on Gangliotetraose

• Mice engineered to lack GM2/GD2 synthase (GalNAc-T), with resultant deficit of GM2, GD2, and all gangliotetraose gangliosides, were originally described as showing a relatively normal phenotype with only a slight reduction in nerve conduction [5].
• Some normal human sera contain a natural IgM antibody against gangliotetraose and GM2 which is capable of initiating complement-mediated cytolysis of HIV-1-infected cells [6].
• Cultured neurons from mice genetically engineered to lack gangliotetraose gangliosides such as GM1 were highly vulnerable to Ca2+-induced apoptosis [7].
• The nuclear envelope (NE), consisting of two unique membranes, is one such structure shown to contain members of the gangliotetraose family and possibly other sialoglycolipids [7].
• The gangliosides of the gangliotetraose series were individually determined with cholera toxin subunit B by TLC-enzyme-linked immunosorbent assay (ELISA) after chromatography and subsequent sialidase hydrolysis to II3NeuAc-GgOse4Cer (GM1) [8].

Chemical compound and disease context of Gangliotetraose

• Gangliosides of 11 different neuroblastoma cell lines, grown to confluence, were extracted and quantified with respect to: (a) total lipid-bound sialic acid, (b) total gangliotetraose family, and (c) GM1 content [9].

Biological context of Gangliotetraose

• Our previous study showed an impaired regulation of Ca(2+) homeostasis in cultured cerebellar granule neurons (CGN) from neonatal mice lacking GM2, GD2 and all gangliotetraose gangliosides, due to disruption of the GM2/GD2 synthase (GalNAc-T) gene [10].

Anatomical context of Gangliotetraose

• All evidence thus points to cultured astrocytes of rat and chick brain containing appreciable gangliosides, most of which are GM3 and GD3 with the majority of the remainder comprising structures other than the gangliotetraose type [11].
• The contents of GA1, GM3, GD3, GM2 and gangliotetraose-series gangliosides in ATL cells were all different, even though all the cells used have a common antigen reactive with monoclonal OKT-4 antibody, indicating that there are several subsets of human inducer/helper T-cells, which possess different metabolism and expression of gangliosides [12].
• Gangliotetraose-series gangliosides, GM1a, GD1a and GD1b, were also found in cultured ATL cells, but were not detected in normal human lymphocytes or the lymphocytes of a patient with ATL [12].
• The pronounced increases in gangliosides belonging to the gangliotetraose family during the neurite outgrowth phase of neuronal differentiation have suggested a functional requirement for these substances related to process extension, arborization, and possibly synaptogenesis [13].
• The gangliotetraose ganglioside-deficient mutant cell line, NG-CR72, had significantly higher basal levels of GM2 in the plasma membrane compared to wild type NG108-15 cells, and this level increased significantly on treatment with dendritogenic agents [14].

Associations of Gangliotetraose with other chemical compounds

• One of them, Chol-1 alpha-a, was isolated and characterized as a trisialoganglioside containing the gangliotetraose backbone in which 1 mol of sialic acid was attached to each of the reducing end galactose, N-acetylgalactosamine and internal galactose residues, respectively [15].
• An immunostaining procedure has been developed for the assay of the gangliotetraose gangliosides and sulfatide in cerebrospinal fluid [16].
• Enhanced susceptibility to kainate-induced seizures, neuronal apoptosis, and death in mice lacking gangliotetraose gangliosides: protection with LIGA 20, a membrane-permeant analog of GM1 [17].

Gene context of Gangliotetraose

• Knock-out (KO) mice lacking gangliotetraose gangliosides attributable to disruption of the gene for GM2/GD2 synthase [GalNAcT (UDP-N-acetylgalactosamine:GM3/GD3 beta-1,4-N-acetylgalactosaminyltransferase; EC 2.4.1.92 [EC])] are revealing key neural functions for the complex gangliosides of brain [17].
• Gangliosides of the gangliotetraose series were individually determined with cholera toxin B-subunit (CT-B) and an anti CT-B monoclonal antibody after chromatography and sialidase hydrolysis to GM1 on high performance thin-layer plates [16].

Analytical, diagnostic and therapeutic context of Gangliotetraose

• Several underivatized mono- and bis-sulfated glycosphingolipids having gangliotriaose or gangliotetraose core structure were analyzed by negative liquid secondary ion mass spectrometry (LSIMS) with high- and low-energy collision-induced dissociation (CID) [18].
• These changes, i.e. several-fold increase in concentration and appearance of the gangliotetraose family, have been observed both in vivo and in neuronal cell cultures [19].

References