Chronic cyclodextrin treatment of murine Niemann-Pick C disease ameliorates neuronal cholesterol and glycosphingolipid storage and disease progression.
BACKGROUND:Niemann-Pick type C (NPC) disease is a fatal neurodegenerative disorder caused most commonly by a defect in the NPC1 protein and characterized by widespread intracellular accumulation of unesterified cholesterol and glycosphingolipids (GSLs). While current treatment therapies are limited,...
Main Authors: | Cristin D Davidson, Nafeeza F Ali, Matthew C Micsenyi, Gloria Stephney, Sophie Renault, Kostantin Dobrenis, Daniel S Ory, Marie T Vanier, Steven U Walkley |
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Format: | Article |
Language: | English |
Published: |
Public Library of Science (PLoS)
2009-09-01
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Series: | PLoS ONE |
Online Access: | http://europepmc.org/articles/PMC2736622?pdf=render |
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