Demonstration of GM1-ganglioside in nervous system in generalized GM1-gangliosidosis using cholera toxin B subunit.

By using cholera toxin B subunit and its antibody, the deposition of GM1-ganglioside in the cerebral cortex and peripheral nerves including Meissner and Auerbach's plexuses in the intestine and other visceral nerves of generalized GM1-gangliosidosis was demonstrated. The GM1-ganglioside was found in the swollen neurons of cerebral cortex and ganglion cells of the peripheral nerves. Electron microscopically, parts of membranous cytoplasmic bodies, and amorphous substances among them, revealed a positive reaction for the cholera toxin staining.