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The ubiquitin proteasome system in neurodegenerative diseases: Sometimes the chicken, sometimes the egg
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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