Structural Basis of Low-pH-Dependent Lysosomal Cholesterol Egress by NPC1 and NPC2

Hongwu Qian, Xuelan Wu, Ximing Du, Xia Yao, Xin Zhao, Joyce Lee, Hongyuan Yang, Nieng Yan

Research output: Contribution to journalArticlepeer-review

1 Scopus citations

Abstract

Structural analyses of NPC1 and its complex with NPC2 reveal the basis for low-pH-dependent cholesterol delivery from the lysosomal lumen to the membrane and provide insights into the effects of more than 200 mutations associated with Niemann-Pick disease type C.

Original languageEnglish (US)
Pages (from-to)98-111.e18
JournalCell
Volume182
Issue number1
DOIs
StatePublished - Jul 9 2020

All Science Journal Classification (ASJC) codes

  • Biochemistry, Genetics and Molecular Biology(all)

Keywords

  • NPC1
  • NPC2
  • Niemann-Pick disease type C
  • cholesterol transport
  • cryo-EM
  • lysosomal cholesterol egress
  • structural biology

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