- Author
-
S. Schipper-Krom
K. Juenemann
A.H. Jansen
A. Wiemhoefer
R. van den Nieuwendijk
D.L. Smith
M.A. Hink
G.P. Bates
H. Overkleeft
H. Ovaa
E. Reits - Year
- 2014
- Title
- Dynamic recruitment of active proteasomes into polyglutamine initiated inclusion bodies
- Journal
- FEBS Letters
- Volume | Issue number
- 588 | 1
- Pages (from-to)
- 151-159
- Document type
- Article
- Faculty
- Faculty of Medicine (AMC-UvA)
Faculty of Science (FNWI) - Institute
- Swammerdam Institute for Life Sciences (SILS)
- Abstract
-
Neurodegenerative disorders such as Huntington's disease are hallmarked by neuronal intracellular inclusion body formation. Whether proteasomes are irreversibly recruited into inclusion bodies in these protein misfolding disorders is a controversial subject. In addition, it has been proposed that the proteasomes may become clogged by the aggregated protein fragments, leading to impairment of the ubiquitin-proteasome system. Here, we show by fluorescence pulse-chase experiments in living cells that proteasomes are dynamically and reversibly recruited into inclusion bodies. As these recruited proteasomes remain catalytically active and accessible to substrates, our results challenge the concept of proteasome sequestration and impairment in Huntington's disease, and support the reported absence of proteasome impairment in mouse models of Huntington's disease.
- URL
- go to publisher's site
- Language
- English
- Persistent Identifier
- https://hdl.handle.net/11245/1.431033
Disclaimer/Complaints regulations
If you believe that digital publication of certain material infringes any of your rights or (privacy) interests, please let the Library know, stating your reasons. In case of a legitimate complaint, the Library will make the material inaccessible and/or remove it from the website. Please Ask the Library, or send a letter to: Library of the University of Amsterdam, Secretariat, Singel 425, 1012 WP Amsterdam, The Netherlands. You will be contacted as soon as possible.