Unraveling the sterol-trafficking defect in Niemann-Pick C disease
Author:
Publisher
Proceedings of the National Academy of Sciences
Subject
Multidisciplinary
Reference10 articles.
1. Reversal of defective lysosomal transport in NPC disease ameliorates liver dysfunction and neurodegeneration in the npc1-/- mouse
2. Purified NPC1 Protein
3. NPC2, the Protein Deficient in Niemann-Pick C2 Disease, Consists of Multiple Glycoforms That Bind a Variety of Sterols
4. NPC2 facilitates bidirectional transfer of cholesterol between NPC1 and lipid bilayers, a step in cholesterol egress from lysosomes
5. Genetic evidence for nonredundant functional cooperativity between NPC1 and NPC2 in lipid transport
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