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

doi:10.1016/j.cell.2020.05.020

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 journal › Article › peer-review

112 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 language	English (US)
Pages (from-to)	98-111.e18
Journal	Cell
Volume	182
Issue number	1
DOIs	https://doi.org/10.1016/j.cell.2020.05.020
State	Published - Jul 9 2020

All Science Journal Classification (ASJC) codes

General Biochemistry, Genetics and Molecular Biology

Keywords

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

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10.1016/j.cell.2020.05.020

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title = "Structural Basis of Low-pH-Dependent Lysosomal Cholesterol Egress by NPC1 and NPC2",

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.",

keywords = "NPC1, NPC2, Niemann-Pick disease type C, cholesterol transport, cryo-EM, lysosomal cholesterol egress, structural biology",

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

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AU - Qian, Hongwu

AU - Wu, Xuelan

AU - Du, Ximing

AU - Yao, Xia

AU - Zhao, Xin

AU - Lee, Joyce

AU - Yang, Hongyuan

AU - Yan, Nieng

PY - 2020/7/9

Y1 - 2020/7/9

N2 - 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.

AB - 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.

KW - NPC1

KW - NPC2

KW - Niemann-Pick disease type C

KW - cholesterol transport

KW - cryo-EM

KW - lysosomal cholesterol egress

KW - structural biology

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JO - Cell

JF - Cell

IS - 1

ER -

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

Abstract

All Science Journal Classification (ASJC) codes

Keywords

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