ganglio-n-triaosylceramide has been researched along with Gangliosidoses* in 2 studies
2 other study(ies) available for ganglio-n-triaosylceramide and Gangliosidoses
| Article | Year |
|---|---|
Recombinant GM2-activator protein stimulates in vivo degradation of GA2 in GM2 gangliosidosis AB variant fibroblasts but exhibits no detectable binding of GA2 in an in vitro assay.
The interaction between glycosphingolipids and recombinant human GM2-activator was studied in a microwell binding assay. A-series gangliosides like GM3, GM2 and GM1 were strongly bound by the recombinant human GM2 activator. A weak binding was observed to GD1b and sulfatide, while neutral glycolipids were not bound. Optimal binding occurred at pH 4.2 and was inhibited by increasing concentrations of citrate buffer and NaCl. In contrast with these in vitro results the recombinant human GM2-activator is able to restore the degradation of GA2 in fibroblasts from patients with the AB variant of GM2 gangliosidosis in vivo. Topics: Biotin; Cells, Cultured; Citrates; Fibroblasts; G(M2) Activator Protein; Gangliosides; Gangliosidoses; Glycosphingolipids; Humans; Hydrogen-Ion Concentration; Infant; Protein Binding; Proteins; Recombinant Proteins; Sandhoff Disease; Sodium Chloride; Tay-Sachs Disease | 1999 |
Characterization of neutral and acidic glycosphingolipids in brains of two patients with GM1 gangliosidosis type 1 and type 2.
Brains of two patients with GM1 gangliosidosis type 1 and type 2, together with the age-matched control brains, were analyzed for glycosphingolipids. Six species of neutral glycolipids, eight species of gangliosides, and sulfatide were isolated from the diseased brains and identified. In addition to GM1 ganglioside and its asialo derivative, the diseased brains accumulated considerable amounts of gangliotriaosylceramide and glycolipids belonging to the globo series, the accumulation of which cannot be explained by deficient beta-galactosidase activity in this disease. GM4 ganglioside was detected in the type 2 brain, but not in type 1. As to fatty acid composition of monohexosylceramides and sulfatide in the two diseased brains, stearic acid was more predominant in the type 1 brain than in the type 2 brain. In light of our previous observations on a Tay-Sachs brain and present results, it appears that metabolism of the globo series glycolipids, which is active in normal brain at early infancy but inactive thereafter, remains in brains with GM1 gangliosidosis (types 1 and 2) and Tay-Sachs disease, reflecting a disturbance in development of the brain. Topics: beta-Galactosidase; Brain; Carbohydrates; Child; Child, Preschool; Fatty Acids; G(M1) Ganglioside; Gangliosides; Gangliosidoses; Glycolipids; Glycosphingolipids; Humans; Male; Sulfoglycosphingolipids | 1985 |