Specific neural cell death in the mouse model of niemann-pick disease type C
β Scribed by Atsuko Yamada; Makoto Saji; Haruaki Ninomiya; Kousaku Ohno
- Book ID
- 117365229
- Publisher
- Elsevier Science
- Year
- 1998
- Tongue
- English
- Weight
- 78 KB
- Volume
- 31
- Category
- Article
- ISSN
- 0168-0102
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π SIMILAR VOLUMES
Niemann-Pick type C (NPC) disease develops as a result of mutations in the NPC1 gene that encodes a protein involved in the net movement of unesterified cholesterol from the late endosomal/lysosomal compartment to the metabolically active pool of sterol in the cytosol of virtually every cell in the
## Abstract NiemannβPick disease type C (NPC) is a progressive neurodegenerative disorder characterized by accumulation of free cholesterol in lysosomes, mainly due to a mutation in the NPC1 gene. The pathophysiological basis of the neural disorders in NPC, however, is not well understood. We found