Niemann-Pick C disease and mobilization of lysosomal cholesterol by cyclodextrin

Niemann-Pick C disease and mobilization of lysosomal cholesterol by cyclodextrin

Niemann-Pick type C (NPC) disease is a lysosomal storage disease in which endocytosed cholesterol becomes sequestered in late endosomes/lysosomes (LEs/Ls) because of mutations in either the NPC1 or NPC2 gene. Mutations in either of these genes can lead to impaired functions of the NPC1 or NPC2 prote...

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Bibliographic Details
Main Authors: Jean E. Vance, Barbara Karten
Format: Article
Language:English
Published: Elsevier 2014-08-01
Series:Journal of Lipid Research
Subjects:
neurodegenerative disease
lysosomal storage disease
Purkinje cells
gangliosides
neurons
astrocytes
Online Access:http://www.sciencedirect.com/science/article/pii/S0022227520353256

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http://www.sciencedirect.com/science/article/pii/S0022227520353256

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