Abstract
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 language | English |
|---|---|
| Title of host publication | Handbook of Neurochemistry and Molecular Neurobiology |
| Subtitle of host publication | Neural Protein Metabolism and Function |
| Publisher | Springer US |
| Pages | 371-408 |
| Number of pages | 38 |
| ISBN (Print) | 9780387303468 |
| DOIs | |
| State | Published - 2007 |