Cholesterol accumulation is associated with lysosomal dysfunction and autophagic stress in Npc1 -/- mouse brain.

@article{Liao2007CholesterolAI,
  title={Cholesterol accumulation is associated with lysosomal dysfunction and autophagic stress in Npc1 -/- mouse brain.},
  author={Guanghong Liao and Yueqin Yao and Jihua Liu and Zhang Yu and Simon T.Y. Cheung and Ang Xie and Xiaoli Liang and Xiaoning Bi},
  journal={The American journal of pathology},
  year={2007},
  volume={171 3},
  pages={962-75}
}
Niemann-Pick type C (NPC) disease is an autosomal recessive disorder caused by mutations of NPC1 and NPC2 genes. Progressive neurodegeneration that accompanies NPC is fatal, but the underlying mechanisms are still poorly understood. In the present study, we characterized the association of autophagic-lysosomal dysfunction with cholesterol accumulation in Npc1(-/-) mice during postnatal development. Brain levels of lysosomal cathepsin D were significantly higher in mutant than in wild-type mice… CONTINUE READING
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