Association of autophagy with cholesterol-accumulated compartments in Niemann-Pick disease type C cells.

@article{Ishibashi2009AssociationOA,
  title={Association of autophagy with cholesterol-accumulated compartments in Niemann-Pick disease type C cells.},
  author={Seiya Ishibashi and Tsuneo Yamazaki and Koichi Okamoto},
  journal={Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia},
  year={2009},
  volume={16 7},
  pages={954-9}
}
Niemann-Pick disease type C (NPC) is an autosomal recessive disease most commonly caused by a mutation of NPC1, resulting in the accumulation of cholesterol in late endosomes or lysosomes. In this study, we examined whether an abnormality of autophagy is involved in the pathogenesis of NPC and how cholesterol accumulation participates in this process, using both a U18666A-induced NPC model and NPC1-deficient Chinese hamster ovary cells. In these cells, an increase in the level of the… CONTINUE READING

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