GD2 ganglioside is an important example of tumour-associated carbohydrate antigens. Over expression of the GD2 ganglioside is a hallmark of neuroblastoma, while its expression on normal cells is restricted. The antigen is stably expressed on cells of the tumour, and retained during a therapy of the disease (Kramer et al., 2001). The facts allow to use its presence to diagnose neuroblastoma, mon...

A procedure is described for the preparation of Tay-Sachs ganglioside specifically labeled in the sialic acid portion of the molecule. Rat brain gangliosides were labeled biosynthetically by the intracranial injection of N-acetyl-(3)H-D-mannosamine. Radioactive gangliosides were isolated and selectively degraded with bacterial neuraminidase and rat liver beta-galactosidase to Tay-Sachs ganglios...

GM1 gangliosidosis is an incurable lysosomal storage disease caused by a deficiency in acid b-galactosidase (b-gal), resulting in accumulation of ganglioside GM1 and its asialo-form (GA1) primarily in the brain. In this study, we investigated the effects of N-butyldeoxygalacto-nojirimycin (NB-DGJ), an imino sugar that inhibits ganglioside biosynthesis, in normal C57BL/6J (B6) and in b-gal knock...

The composition, structure and localization of gangliosides of aorta taken from subjects who had died after myocardial infarction were studied. Individual gangliosides were purified by high-performance liquid chromatography and high-performance thin-layer chromatography and were characterized on the basis of their chromatographic mobility, carbohydrate composition, neuraminidase hydrolysis and ...

Botulinum neurotoxins are highly toxic, and bind two receptors to achieve their high affinity and specificity for neurons. Here we present the first structure of a botulinum neurotoxin bound to both its receptors. We determine the 2.3-Å structure of a ternary complex of botulinum neurotoxin type B bound to both its protein receptor synaptotagmin II and its ganglioside receptor GD1a. We show tha...

As evidenced by their ability to reduce cerebral edema, exogenous ganglioside administration exerts acute effects on CNS injury processes. We report here that ganglioside (GM1 or AGF2) treatment results in a 52% decrease in mortality 48 hours after the induction of ischemia in gerbils by permanent unilateral ligation of the common carotid artery. By comparing the occluded vs. nonoccluded sides ...

A binding protein specific for major neuronal gangliosides was detected on rat brain membranes using a synthetic ganglioside-protein conjugate, 125I-(GT1b)4BSA (bovine serum albumin derivatized with 4 mol of ganglioside GT1b/mol of protein), as a radioligand. Specific binding of the ligand displayed marked regional variation within the brain, with white matter-enriched regions demonstrating the...

Anti-idiotypic MAb were raised in syngeneic mice against a mouse MAb recognizing GD3 ganglioside (MAb R24). Two anti-idiotypic MAb, designated BEC2 and BEC3, recognized distinct determinants on MAb R24 that mapped near or within the GD3-binding site. New Zealand white rabbits, which express GD3 on normal tissues, were immunized with either BEC2, BEC3, or control MAb FLOPC-21. All rabbits develo...

Gangliosides were extracted from purified human and porcine thyrotropin (TSH) receptors (TSH-R) and were detected by probing with an 125I-labeled sialic acid-specific lectin, Limax flavus agglutinin. Gangliosides copurified with human and porcine TSH-R migrated between monosialoganglioside GM1 and disialoganglioside GD1a. Ceramide glycanase digestion of the purified human TSH-R-associated glyco...

The effect of difluoromethylornithine (DFMO), an irreversible inhibitor of ornithine decarboxylase (ODC), the rate limiting enzyme of polyamine biosynthesis, and its combined action with GM1 ganglioside, was studied on the GFAP content in a model of remote astrogliosis evoked in the hippocampus by lateral fimbria transection. DFMO markedly suppressed hippocampal gliosis as measured by GFAP immu...