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.
D002540:Cerebral Cortex / Q000378:metabolism*; D002772:Cholera Toxin / Q000378:metabolism; D005260:Female; D005677:G(M1) Ganglioside / Q000378:metabolism*; D005733:Gangliosidoses / Q000378:metabolism*; D006801:Humans; D008854:Microscopy, Electron; D009197:Myenteric Plexus / Q000378:metabolism; D010179:Pancreas / Q000294:innervation; D010446:Peptide Fragments / Q000378:metabolism; D010525:Peripheral Nerves / Q000378:metabolism*; D013368:Submucous Plexus / Q000378:metabolism
D010446:Peptide Fragments; D005677:G(M1) Ganglioside; C050310:cholera toxin, B subunit (50-64); D002772:Cholera Toxin