Development of a Rab9 Transgenic Mouse and Its Ability to Increase the Lifespan of a Murine Model of Niemann-Pick Type C Disease
β Scribed by Tatiana Kaptzan; Sally A. West; Eileen L. Holicky; Christine L. Wheatley; David L. Marks; Tengke Wang; Kyle B. Peake; Jean Vance; Steven U. Walkley; Richard E. Pagano
- Book ID
- 117970888
- Publisher
- American Society for Investigative Pathology
- Year
- 2009
- Tongue
- English
- Weight
- 728 KB
- Volume
- 174
- Category
- Article
- ISSN
- 0002-9440
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## Abstract NiemannβPick 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 endosomalβlysosomal system. Most studies on the mechanisms of neurodegenera
## 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