Protein degradation and protection against misfolded or damaged proteins
Top Cited Papers
- 1 December 2003
- journal article
- review article
- Published by Springer Nature in Nature
- Vol. 426 (6968) , 895-899
- https://doi.org/10.1038/nature02263
Abstract
The ultimate mechanism that cells use to ensure the quality of intracellular proteins is the selective destruction of misfolded or damaged polypeptides. In eukaryotic cells, the large ATP-dependent proteolytic machine, the 26S proteasome, prevents the accumulation of non-functional, potentially toxic proteins. This process is of particular importance in protecting cells against harsh conditions (for example, heat shock or oxidative stress) and in a variety of diseases (for example, cystic fibrosis and the major neurodegenerative diseases). A full understanding of the pathogenesis of the protein-folding diseases will require greater knowledge of how misfolded proteins are recognized and selectively degraded.Keywords
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