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Differential response of the liver to bile acid treatment in a mouse model of Niemann-Pick disease type C

Abstract:

Niemann-Pick disease type C (NPC) disease is a neurodegenerative lysosomal storage disease caused by mutations in the NPC1 or NPC2 genes. Liver disease is also a common feature of NPC that can present as cholestatic jaundice in the neonatal period. Liver enzymes can remain elevated above the normal range in some patients as they age. We recently reported suppression of the P450 detoxification system in a mouse model of NPC disease and in post-mortem liver from NPC patients. As bile acids regu...

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Publication status:
Published
Peer review status:
Peer reviewed
Version:
Publisher's version

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Publisher copy:
10.12688/wellcomeopenres.12431.2

Authors


More by this author
Institution:
University of Oxford
Division:
Medical Sciences Division
Department:
Pharmacology
More by this author
Institution:
University of Oxford
Division:
Medical Sciences Division
Department:
Pharmacology
More by this author
Institution:
University of Oxford
Division:
Medical Sciences Division
Department:
Pharmacology
More by this author
Institution:
University of Oxford
Division:
Medical Sciences Division
Department:
Pharmacology
Oxford college:
Merton College
Action Medical Research More from this funder
Niemann-Pick Research Fundation More from this funder
Royal Society More from this funder
Publisher:
F1000Research Publisher's website
Journal:
Wellcome Open Research Journal website
Volume:
2
Pages:
76
Publication date:
2017-08-31
Acceptance date:
2017-08-31
DOI:
EISSN:
2398-502X
ISSN:
2398-502X
Pubs id:
pubs:742539
URN:
uri:d71b29cf-e005-4f73-b6f0-10c8be0c91b2
UUID:
uuid:d71b29cf-e005-4f73-b6f0-10c8be0c91b2
Local pid:
pubs:742539

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