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Journal article

FTY720/fingolimod increases NPC1 and NPC2 expression and reduces cholesterol and sphingolipid accumulation in Niemann-Pick type C mutant fibroblasts

Abstract:

Niemann-Pick type C (NPC) disease is a fatal neurodegenerative disorder caused by mutations in NPC1 or NPC2 with decreased functions leading to lysosomal accumulation of cholesterol and sphingolipids. FTY720/fingolimod, used for treatment of multiple sclerosis, is phosphorylated by nuclear sphingosine kinase 2 and its active phosphorylated form (FTY720-P) is an inhibitor of class I histone deacetylases. In this study, administration of clinically relevant doses of FTY720 to mice increased exp...

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Publication status:
Published
Peer review status:
Peer reviewed

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Publisher copy:
10.1096/fj.201601041R

Authors


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Institution:
University of Oxford
Division:
MSD
Department:
Pharmacology
Role:
Author
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Funding agency for:
Platt, F
Grant:
Investigator in Science
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Funding agency for:
Platt, F
Grant:
Investigator in Science
More from this funder
Funding agency for:
Platt, F
Grant:
Investigator in Science
Publisher:
Federation of American Societies for Experimental Biology Publisher's website
Journal:
FASEB Journal Journal website
Volume:
31
Issue:
4
Pages:
1719-1730
Publication date:
2017-01-12
Acceptance date:
2017-01-03
DOI:
ISSN:
1530-6860
Source identifiers:
671696
Language:
English
Keywords:
Pubs id:
pubs:671696
UUID:
uuid:3edb48f2-bd72-40af-847e-990c1a1e0f2d
Local pid:
pubs:671696
Deposit date:
2017-01-30

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