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Journal article

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

Abstract:

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 onset and progression of neuropathological insults in NPC we have systematically examined the CNS of a mouse model of NPC1 (Npc1(-/-) mice) at different stages of the disease course. This revealed a...

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Publisher copy:
10.1016/j.nbd.2011.12.027

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Institution:
University of Oxford
Department:
Oxford, MSD, Pharmacology
Role:
Author
Journal:
Neurobiology of disease
Volume:
45
Issue:
3
Pages:
1086-1100
Publication date:
2012-03-05
DOI:
EISSN:
1095-953X
ISSN:
0969-9961
URN:
uuid:da14a291-409b-405f-9db3-a65407b9448a
Source identifiers:
313914
Local pid:
pubs:313914

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