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  4. Cyclodextrin triggers MCOLN1-dependent endo-lysosome secretion in Niemann-Pick type C cells[S]
 
research article

Cyclodextrin triggers MCOLN1-dependent endo-lysosome secretion in Niemann-Pick type C cells[S]

Vacca, Fabrizio
•
Vossio, Stefania
•
Mercier, Vincent
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April 1, 2019
Journal Of Lipid Research

In specialized cell types, lysosome-related organelles support regulated secretory pathways, whereas in nonspecialized cells, lysosomes can undergo fusion with the plasma membrane in response to a transient rise in cytosolic calcium. Recent evidence also indicates that lysosome secretion can be controlled transcriptionally and promote clearance in lysosome storage diseases. In addition, evidence is also accumulating that low concentrations of cyclodextrins reduce the cholesterol-storage phenotype in cells and animals with the cholesterol storage disease Niemann-Pick type C, via an unknown mechanism. Here, we report that cyclodextrin triggers the secretion of the endo/lysosomal content in nonspecialized cells and that this mechanism is responsible for the decreased cholesterol overload in Niemann-Pick type C cells. We also find that the secretion of the endo/lysosome content occurs via a mechanism dependent on the endosomal calcium channel mucolipin-1, as well as FYCO1, the AP1 adaptor, and its partner Gadkin. We conclude that endo-lysosomes in nonspecialized cells can acquire secretory functions elicited by cyclodextrin and that this pathway is responsible for the decrease in cholesterol storage in Niemann-Pick C cells.

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Type
research article
DOI
10.1194/jlr.M089979
Web of Science ID

WOS:000466567300015

Author(s)
Vacca, Fabrizio
Vossio, Stefania
Mercier, Vincent
Moreau, Dimitri
Johnson, Shem
Scott, Cameron C.
Montoya, Jonathan Paz
Moniatte, Marc
Gruenberg, Jean
Date Issued

2019-04-01

Published in
Journal Of Lipid Research
Volume

60

Issue

4

Start page

832

End page

843

Subjects

Biochemistry & Molecular Biology

•

cellular cholesterol

•

cholesterol

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trafficking

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endocytosis

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niemann-pick disease

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phospholipids

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lysobisphosphatidic acid

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bis(monoacylglycero)phosphate

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endosomes

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secretory lysosomes

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disease type-c

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plasma-membrane

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cholesterol transfer

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sterol binding

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transport

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npc1

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exocytosis

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protein

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identification

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quantitation

Editorial or Peer reviewed

REVIEWED

Written at

EPFL

EPFL units
PTECH  
Available on Infoscience
June 18, 2019
Use this identifier to reference this record
https://infoscience.epfl.ch/handle/20.500.14299/157175
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