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Prion degradation pathways: Potential for therapeutic intervention.

Rob Goold1, Chris McKinnon1, Sarah J Tabrizi1

  • 1Department of Neurodegenerative Disease, UCL Institute of Neurology, University College London, United Kingdom.

Molecular and Cellular Neurosciences
|January 14, 2015
PubMed
Summary

Prion diseases involve misfolded proteins accumulating in the brain. Targeting cellular pathways to degrade these abnormal prion proteins (PrPSc) shows promise for future treatments.

Keywords:
AutophagyLysosomal degradationPrP(Sc)Prion diseaseProteasomeTherapeutics

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Area of Science:

  • Neuroscience
  • Molecular Biology
  • Protein Chemistry

Background:

  • Prion diseases are fatal neurodegenerative conditions.
  • Disease pathology is associated with the misfolding of cellular prion protein (PrPC) into the abnormal Prion Protein Scrapie (PrPSc).
  • PrPSc accumulates in the brain during disease progression.

Purpose of the Study:

  • To describe cellular pathways mediating PrPSc degradation.
  • To review potential therapeutic targets for prion disease intervention.

Main Methods:

  • Review of existing literature on prion protein degradation pathways.
  • Analysis of cellular mechanisms involved in PrPSc clearance.

Main Results:

  • Identification of specific cellular pathways involved in PrPSc degradation.
  • Discussion of the potential of targeting these pathways for therapeutic strategies.

Conclusions:

  • Strategies stimulating PrPSc degradation show efficacy in experimental models.
  • Further research into these cellular pathways may lead to novel treatments for prion diseases.