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

Ciechanover, A and Brundin, Patrik LU (2003) In Neuron 40(2). p.427-446
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... (More)
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. (Less)
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author
and
organization
publishing date
type
Contribution to journal
publication status
published
subject
in
Neuron
volume
40
issue
2
pages
427 - 446
publisher
Cell Press
external identifiers
  • wos:000185875800017
  • pmid:14556719
  • scopus:0141987860
ISSN
0896-6273
language
English
LU publication?
yes
additional info
The information about affiliations in this record was updated in December 2015. The record was previously connected to the following departments: Neuronal Survival (013212041)
id
085a2e9e-897d-4753-8072-91d0e7811426 (old id 298603)
alternative location
http://www.neuron.org/content/article/abstract?uid=PIIS0896627303006068
date added to LUP
2016-04-01 12:04:07
date last changed
2022-04-29 00:07:02
@article{085a2e9e-897d-4753-8072-91d0e7811426,
  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.}},
  author       = {{Ciechanover, A and Brundin, Patrik}},
  issn         = {{0896-6273}},
  language     = {{eng}},
  number       = {{2}},
  pages        = {{427--446}},
  publisher    = {{Cell Press}},
  series       = {{Neuron}},
  title        = {{The ubiquitin proteasome system in neurodegenerative diseases: Sometimes the chicken, sometimes the egg}},
  url          = {{http://www.neuron.org/content/article/abstract?uid=PIIS0896627303006068}},
  volume       = {{40}},
  year         = {{2003}},
}