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

      1 ,
      Neuron
      Elsevier BV

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          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.

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          Author and article information

          Journal
          Neuron
          Neuron
          Elsevier BV
          0896-6273
          0896-6273
          Oct 09 2003
          : 40
          : 2
          Affiliations
          [1 ] Department of Biochemistry and The Rappaport Family Institute for Research in the Medical Sciences, The Bruce Rappaport Faculty of Medicine, Technion-Israel Institute of Technology, Haifa 31096, Israel. c_tzachy@netvision.net.il
          Article
          S0896627303006068
          10.1016/s0896-6273(03)00606-8
          14556719
          bafa23b2-87a0-4200-bed6-d9a14197672a
          History

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