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Related Experiment Video

Updated: Jul 4, 2026

High-throughput Screening for Protein-based Inheritance in S. cerevisiae
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High-throughput Screening for Protein-based Inheritance in S. cerevisiae

Published on: August 8, 2017

The prion's elusive reason for being.

Adriano Aguzzi1, Frank Baumann, Juliane Bremer

  • 1Institute of Neuropathology, University of Zurich, CH-8091 Zurich, Switzerland. Adriano.Aguzzi@usz.ch

Annual Review of Neuroscience
|June 19, 2008
PubMed
Summary

Prion diseases are caused by infectious proteins called prions, lacking nucleic acids. Understanding the normal cellular prion protein (PrP(C)) function may unlock the mechanisms behind neurodegeneration in these conditions.

Area of Science:

  • Neuroscience
  • Biochemistry
  • Molecular Biology

Background:

  • Transmissible spongiform encephalopathies (TSEs) are linked to the protein-only hypothesis, involving prions.
  • Prions are infectious agents composed of protein, specifically the misfolded prion protein (PrP(Sc)), an isoform of the normal cellular prion protein (PrP(C)).
  • Despite extensive research, the normal function of PrP(C) and the molecular basis of neurodegeneration in prion diseases remain elusive.

Purpose of the Study:

  • To review the characteristics of prion diseases.
  • To explore the potential role of PrP(C) physiological functions in TSE pathogenesis.
  • To elucidate the toxic mechanisms underlying neurodegeneration in prion-related disorders.

Main Methods:

  • Review of existing scientific literature on prion diseases and the prion protein.

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Protein Misfolding Cyclic Amplification of Prions
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Protein Misfolding Cyclic Amplification of Prions

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Related Experiment Videos

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Protein Misfolding Cyclic Amplification of Prions
10:12

Protein Misfolding Cyclic Amplification of Prions

Published on: November 7, 2012

  • Analysis of the structural and functional properties of PrP(C) and PrP(Sc).
  • Correlation of PrP(C) functions with observed neurotoxic phenotypes in prion diseases.

    • Main Results:

    • Prion diseases are characterized by the accumulation of misfolded PrP(Sc) isoforms.
    • The normal function of PrP(C) is critical for understanding disease mechanisms.
    • Insights into PrP(C) function may explain neurodegeneration in prion disorders.

    Conclusions:

    • The protein-only hypothesis remains a central concept in prion disease research.
    • Further investigation into PrP(C) physiological roles is essential for therapeutic development.
    • Understanding PrP(C) function is key to deciphering neurodegenerative pathways in prion diseases.