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From neuronal inclusions to neurodegeneration: neuropathological investigation of a transgenic mouse model of Huntington'sdisease

✍ Scribed by Davies, S. W.; Mark, T.; Cozens, B. A.; Raza, A. S.; Mahal, A.; Mangiarini, L.; Bates, G. P.


Book ID
120152758
Publisher
The Royal Society
Year
1999
Tongue
English
Weight
571 KB
Volume
354
Category
Article
ISSN
0080-4622

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## Abstract Niemann‐Pick disease type C (NPC) is a deadly neurodegenerative disease often caused by mutation in a gene called NPC1, which results in the accumulation of unesterified cholesterol and glycosphingolipids in the endosomal–lysosomal system. Most studies on the mechanisms of neurodegenera