Chronic cyclodextrin treatment of murine Niemann-Pick C disease ameliorates neuronal cholesterol and glycosphingolipid storage and disease progression.

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,...

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Bibliographic Details
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
Format: Article
Language:English
Published: Public Library of Science (PLoS) 2009-09-01
Series:PLoS ONE
Online Access:http://europepmc.org/articles/PMC2736622?pdf=render

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http://europepmc.org/articles/PMC2736622?pdf=render

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