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Journal article

Impaired antibacterial autophagy links granulomatous intestinal inflammation in Niemann-Pick disease type C1 and XIAP deficiency with NOD2 variants in Crohn's disease.

Abstract:

Objective

Patients with Niemann–Pick disease type C1 (NPC1), a lysosomal lipid storage disorder that causes neurodegeneration and liver damage, can present with IBD, but neither the significance nor the functional mechanism of this association is clear. We studied bacterial handling and antibacterial autophagy in patients with NPC1.

Design

We characterised intestinal inflammation in 14 patients with NPC1 who developed IBD. We investigated bacterial handling and cyt...

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Publication status:
Published
Peer review status:
Peer reviewed

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Publisher copy:
10.1136/gutjnl-2015-310382

Authors


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Institution:
University of Oxford
Division:
MSD
Department:
NDM
Sub department:
NDM Experimental Medicine
Role:
Author
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Institution:
University of Oxford
Division:
MSD
Department:
NDM
Sub department:
NDM Experimental Medicine
Role:
Author
More by this author
Institution:
University of Oxford
Division:
MSD
Department:
NDM
Sub department:
Oxford Ludwig Institute
Role:
Author
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Funding agency for:
Schwerd, T
Grant:
SCHW1730/1-1
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Funding agency for:
Gyrd-Hansen, M
Grant:
102894/Z/13/Z
More from this funder
Funding agency for:
Gyrd-Hansen, M
Grant:
102894/Z/13/Z
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Publisher:
BMJ Publishing Group Publisher's website
Journal:
Gut Journal website
Publication date:
2016-03-01
Acceptance date:
2016-01-14
DOI:
EISSN:
1468-3288
ISSN:
0017-5749
Source identifiers:
609214
Language:
English
Keywords:
Pubs id:
pubs:609214
UUID:
uuid:068f032d-262e-46b8-bf81-3e023839b419
Local pid:
pubs:609214
Deposit date:
2016-03-26

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