The proteasome, protein aggregation, and neurodegeneration

Research output: Chapter in Book/Report/Conference proceedingChapterpeer-review


The presence of intracellular inclusions containing aggregated proteins feature in affected neurons of patients with neurodegenerative disease. Such inclusions also contain immunoreactive ubiquitin and proteasome subunits. Similar perinuclear inclusions termed aggresomes are formed when cells are treated with proteasome inhibitors. Since unfolded proteins are predominantly degraded by the ubiquitin-proteasome system (UPS), a dysfunction of this system has been proposed to contribute to neurodegeneration. This chapter presents an outline of the biochemistry of the UPS followed by an examination of the experimental evidence relating this protein-degrading system to Parkinson's disease, to protein aggregation and aggresome formation, and to polyglutamine disease.

Original languageEnglish
Title of host publicationHandbook of Neurochemistry and Molecular Neurobiology
Subtitle of host publicationNeural Protein Metabolism and Function
PublisherSpringer US
Number of pages38
ISBN (Print)9780387303468
StatePublished - 2007


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