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Miglustat Improves Purkinje Cell Survival and Alters Microglial Phenotype in Feline Niemann-Pick Disease Type C

Niemann-Pick disease type C (NPC disease) is an incurable cellular lipid trafficking disorder characterized by neurodegeneration and intralysosomal accumulation of cholesterol and glycosphingolipids. Treatment with miglustat, a small imino sugar that reversibly inhibits glucosylceramide synthase, wh...

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Bibliographic Details
Main Authors: Stein, Veronika M., Crooks, Alexandra, Ding, Wenge, Prociuk, Maria, O’Donnell, Patricia, Bryan, Caroline, Sikora, Tracey, Dingemanse, Jasper, Vanier, Marie T., Walkley, Steven U., Vite, Charles H.
Format: Artigo
Language:Inglês
Published: 2012
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Online Access:https://ncbi.nlm.nih.gov/pmc/articles/PMC3352323/
https://ncbi.nlm.nih.gov/pubmed/22487861
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1097/NEN.0b013e31825414a6
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