Proteasome Activator Enhances Survival of Huntington's Disease Neuronal Model Cells

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Proteasome Activator Enhances Survival of Huntington's Disease Neuronal Model Cells

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Title: Proteasome Activator Enhances Survival of Huntington's Disease Neuronal Model Cells
Author: Kim, Woori; Cattaneo, Elena; Seo, Hyemyung; Sonntag, Kai-Christian; Isacson, Ole Stefan

Note: Order does not necessarily reflect citation order of authors.

Citation: Seo, Hyemyung, Kai-Christian Sonntag, Woori Kim, Elena Cattaneo, and Ole Isacson. 2007. Proteasome activator enhances survival of Huntington's Disease neuronal model cells. PLoS ONE 2(2): e238.
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Abstract: In patients with Huntington's disease (HD), the proteolytic activity of the ubiquitin proteasome system (UPS) is reduced in the brain and other tissues. The pathological hallmark of HD is the intraneuronal nuclear protein aggregates of mutant huntingtin. We determined how to enhance UPS function and influence catalytic protein degradation and cell survival in HD. Proteasome activators involved in either the ubiquitinated or the non-ubiquitinated proteolysis were overexpressed in HD patients' skin fibroblasts or mutant huntingtin-expressing striatal neurons. Following compromise of the UPS, overexpression of the proteasome activator subunit PA28γ, but not subunit S5a, recovered proteasome function in the HD cells. PA28γ also improved cell viability in mutant huntingtin-expressing striatal neurons exposed to pathological stressors, such as the excitotoxin quinolinic acid and the reversible proteasome inhibitor MG132. These results demonstrate the specific functional enhancements of the UPS that can provide neuroprotection in HD cells.
Published Version: doi:10.1371/journal.pone.0000238
Other Sources: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1800909/pdf/
Terms of Use: This article is made available under the terms and conditions applicable to Other Posted Material, as set forth at http://nrs.harvard.edu/urn-3:HUL.InstRepos:dash.current.terms-of-use#LAA
Citable link to this page: http://nrs.harvard.edu/urn-3:HUL.InstRepos:4911699

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