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Iminosugar-Based Inhibitors of Glucosylceramide Synthase Increase Brain Glycosphingolipids and Survival in a Mouse Model of Sandhoff Disease

The neuropathic glycosphingolipidoses are a subgroup of lysosomal storage disorders for which there are no effective therapies. A potential approach is substrate reduction therapy using inhibitors of glucosylceramide synthase (GCS) to decrease the synthesis of glucosylceramide and related glycosphin...

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Detalles Bibliográficos
Autores principales:	Ashe, Karen M., Bangari, Dinesh, Li, Lingyun, Cabrera-Salazar, Mario A., Bercury, Scott D., Nietupski, Jennifer B., Cooper, Christopher G. F., Aerts, Johannes M. F. G., Lee, Edward R., Copeland, Diane P., Cheng, Seng H., Scheule, Ronald K., Marshall, John
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	Public Library of Science 2011
Materias:	Research Article
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3126858/ https://www.ncbi.nlm.nih.gov/pubmed/21738789 http://dx.doi.org/10.1371/journal.pone.0021758

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