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| The ubiquitin proteasome system in neurodegenerative diseases: Sometimes the chicken, sometimes the egg |
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| Author(s): Ciechanover A, Brundin P |
| Source: NEURON Volume: 40 Issue: 2 Pages: 427-446 Published: OCT 9 2003 |
| Times Cited: 273 References: 163 |
| Abstract: The ubiquitin-proteasome system targets numerous cellular proteins for degradation. In addition, modifications by ubiquitin-like proteins as well as proteins containing ubiquitin-interacting and -associated motifs modulate many others. This tightly controlled process involves multiple specific and general enzymes of the system as well as many modifying and ancillary proteins. Thus, it is not surprising that ubiquitin-mediated degradation/processing/modification regulates a broad array of basic cellular processes. Moreover, aberrations in the system have been implicated, either as a primary cause or secondary consequence, in the pathogenesis of both inherited and acquired neurodegenerative diseases. Recent findings indicate that the system is involved in the pathogenesis of Parkinson's, Alzheimer's, Huntington's, and Prion diseases as well as amyotrophic lateral sclerosis. This raises hopes for a better understanding of the pathogenetic mechanisms involved in these diseases and for the development of novel, mechanism-based therapeutic modalities. |
| Document Type: Review |
| Language: English |
| Reprint Address: Ciechanover, A (reprint author), Technion Israel Inst Technol, Bruce Rappaport Fac Med, Dept Biochem, IL-31096 Haifa, Israel |
Addresses:
1. Technion Israel Inst Technol, Bruce Rappaport Fac Med, Dept Biochem, IL-31096 Haifa, Israel 2. Technion Israel Inst Technol, Bruce Rappaport Fac Med, Rappaport Family Inst Res Med Sci, IL-31096 Haifa, Israel 3. Lund Univ, Dept Physiol Sci, Sect Neuronal Survival, Wallenberg Neurosci Ctr,Biomed Ctr, S-22184 Lund, Sweden |
| Publisher: CELL PRESS, 1100 MASSACHUSETTS AVE, CAMBRIDGE, MA 02138 USA |
| Subject Category: Neurosciences |
| IDS Number: 731GK |
| ISSN: 0896-6273 |
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| |  |  |  |  | | | | Record from Web of Science® | |  |  | | | | | | |