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Protein Misfolding and Axonal Protection in Neurodegenerative Diseases

  • Haruhisa Inoue
  • Takayuki Kondo
  • Ling Lin
  • Sha Mi
  • Ole Isacson
  • Ryosuke Takahashi
Chapter
Part of the Focus on Structural Biology book series (FOSB, volume 7)

Abstract

Genetically engineered mouse model studies show that neuronal dysfunction caused by protein aggregation/misfolding are reversible, indicating that injured neurons are alive even under disease states. Protein misfolding/aggregation in axons and distal dominant axonal degeneration are observed in a subgroup of degenerative diseases and in certain experimental conditions. Moreover, therapeutic approaches towards axonal protection are effective in neurodegenerative disease mouse models; (a) axonal regeneration, (b) anti-Wallerian degeneration, (c) autophagy enhancement, and (d) stabilization of microtubules. These studies demonstrate that axonal protection/functional repair of axons can be general therapeutic interventions for neurodegenerative diseases.

Keywords

Amyotrophic Lateral Sclerosis Amyotrophic Lateral Sclerosis Patient Axonal Regeneration Axonal Degeneration Neuronal Dysfunction 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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Copyright information

© Springer Science+Business Media B.V. 2009

Authors and Affiliations

  • Haruhisa Inoue
    • 1
  • Takayuki Kondo
  • Ling Lin
  • Sha Mi
  • Ole Isacson
  • Ryosuke Takahashi
  1. 1.Department of NeurologyKyoto University Graduate School of MedicineSakyo-kuJapan

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