- 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...
Expand abstract - Publisher copy:
- 10.1016/j.nbd.2011.12.027
-
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- 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
- Language:
- English
- Keywords:
- Copyright date:
- 2012
Journal article
Early glial activation, synaptic changes and axonal pathology in the thalamocortical system of Niemann-Pick type C1 mice.
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