Early glial activation, synaptic changes and axonal pathology in the thalamocortical system of Niemann–Pick type C1 mice

Niemann–Pick disease type C (NPC) is an inherited lysosomal storage disease characterised by accumulation of cholesterol and glycosphingolipids. NPC patients suffer a progressive neurodegenerative phenotype presenting with motor dysfunction, mental retardation and cognitive decline. To examine the o...

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Bibliographic Details
Main Authors: Sarah N.R. Pressey, David A. Smith, Andrew M.S. Wong, Frances M. Platt, Jonathan D. Cooper
Format: Article
Language:English
Published: Elsevier 2012-03-01
Series:Neurobiology of Disease
Subjects:
Online Access:http://www.sciencedirect.com/science/article/pii/S0969996111004025