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Neuroprotective activity of ursodeoxycholic acid in CHMP2B Intron5 models of frontotemporal dementia

Authors
  • West, Ryan J.H.1, 2
  • Ugbode, Chris3
  • Fort-Aznar, Laura3
  • Sweeney, Sean T.3
  • 1 Sheffield Institute for Translational Neuroscience, University of Sheffield, S10 2HQ, UK
  • 2 Neuroscience Institute, University of Sheffield, Western Bank, Sheffield S10 2TN, UK
  • 3 Department of Biology, University of York, York YO10 5DD, UK
Type
Published Article
Journal
Neurobiology of Disease
Publisher
Elsevier
Publication Date
Oct 01, 2020
Volume
144
Identifiers
DOI: 10.1016/j.nbd.2020.105047
PMID: 32801000
PMCID: PMC7491204
Source
PubMed Central
Keywords
License
Unknown

Abstract

• Defective endosomal proteostasis is a hallmark of CHMP2BIntron5 FTD/ALS. • CHMP2BIntron5 causes neuronal dysfunction in Drosophila and mammalian models. • FDA approved compound UDCA rescues neuronal dysfunction in both models. • UDCA treatment does not rescue endosome function or excess ubiquitinated protein. • UDCA treatment highlights glutathione as a novel regulator of CHMP2BIntron5 pathology.

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