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Utilizing a specific inactivator of the lysosomal enzyme GM<jats:sub>1<\/jats:sub>\u2010ganglioside \u03b2\u2010galactosidase (\u03b2\u2010D\u2010galactopyranosylmethyl\u2010<jats:italic>p<\/jats:italic>\u2010nitrophenyltriazene [\u03b2\u2010GalMNT]) and neuroblastoma X glioma hybrid cells (NG108\u201315), we suppressed \u03b2\u2010galactosidase activity for up to 72 hours. Coincidental with suppression of this enzyme to levels less than 1% of control, we found up to a nine\u2010fold accumulation of its substrate, the GM<jats:sub>1<\/jats:sub>\u2010ganglioside, and the ultrastructural appearance of membranous cytoplasmic bodies. \u03b2\u2010GalMNT treatment suppressed growth but had little effect on the specific activity of choline acetyltransferase, lactate dehydrogenase, or other lysosomal enzymes including galactosylceramidase. This model should permit studies of the neurophysiological effects of increased ganglioside accumulation and their reversibility.<\/jats:p>","DOI":"10.1002\/ana.410210514","type":"journal-article","created":{"date-parts":[[2005,1,1]],"date-time":"2005-01-01T17:41:33Z","timestamp":1104601293000},"page":"497-503","source":"Crossref","is-referenced-by-count":3,"title":["Inactivation of GM<sub>1<\/sub>\u2010ganglioside \u03b2\u2010galactosidase by a specific inhibitor: A model for ganglioside storage disease"],"prefix":"10.1002","volume":"21","author":[{"given":"Harvey S.","family":"Singer","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Michael","family":"Tiemeyer","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Paul A.","family":"Slesinger","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Michael 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