Article
Cholesterol accumulation sequesters Rab9 and disrupts late endosome function in NPC1-deficient cells.
The Journal of biological chemistry - 30 Jun 2006
Ganley Ian G, Pfeffer Suzanne R
Abstract excerpt
Niemann-Pick type C disease is an autosomal recessive disorder that leads to massive accumulation of cholesterol and glycosphingolipids in late endosomes and lysosomes. To understand how cholesterol accumulation influences late endosome function, we investigated the effect of elevated cholesterol on Rab9-dependent export of mannose 6-phosphate receptors from this compartment. Endogenous Rab9 levels were elevated...
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