The aggravating role of the ubiquitin–proteasome system in neurodegeneration
- 11 January 2005
- journal article
- review article
- Published by Wiley in FEBS Letters
- Vol. 579 (3) , 571-576
- https://doi.org/10.1016/j.febslet.2004.12.058
Abstract
Association of protein inclusions or aggregates within brain tissues of patients with neurodegenerative disorders has been widely reported. These inclusions are commonly characterised both by the presence of ubiquitylated proteins and the sequestration of components of the ubiquitin-proteasome system (UPS). Such observations have led to the proposition that the UPS has a direct role in their formation. Indeed, the presence of ubiquitylated proteins and UPS components in inclusions may reflect unsuccessful attempts by the UPS to remove aggregating proteins. Whether the physical presence of inclusions causes cell death or, conversely, whether they are non-toxic and their presence reflects a cellular protective mechanism remains highly controversial.Keywords
This publication has 61 references indexed in Scilit:
- Systemic exposure to proteasome inhibitors causes a progressive model of Parkinson's diseaseAnnals of Neurology, 2004
- Siah-1 Facilitates Ubiquitination and Degradation of Synphilin-1Journal of Biological Chemistry, 2003
- Parkin-deficient Mice Exhibit Nigrostriatal Deficits but Not Loss of Dopaminergic NeuronsJournal of Biological Chemistry, 2003
- A superfamily of protein tags: ubiquitin, SUMO and related modifiersPublished by Elsevier ,2003
- Conjugation and deconjugation of ubiquitin regulating the destiny of proteinsExperimental & Molecular Medicine, 2003
- The UCH-L1 Gene Encodes Two Opposing Enzymatic Activities that Affect α-Synuclein Degradation and Parkinson's Disease SusceptibilityCell, 2002
- Naturally secreted oligomers of amyloid β protein potently inhibit hippocampal long-term potentiation in vivoNature, 2002
- Inherent toxicity of aggregates implies a common mechanism for protein misfolding diseasesNature, 2002
- Ubiquitination of a New Form of α-Synuclein by Parkin from Human Brain: Implications for Parkinson's DiseaseScience, 2001
- Pathological lesions of Alzheimer's disease and dementia with Lewy bodies brains exhibit immunoreactivity to an ATPase that is a regulatory subunit of the 26S proteasomeNeuroscience Letters, 1996