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Related Experiment Video

Updated: Jul 5, 2026

Investigating the Spreading and Toxicity of Prion-like Proteins Using the Metazoan Model Organism C. elegans
12:57

Investigating the Spreading and Toxicity of Prion-like Proteins Using the Metazoan Model Organism C. elegans

Published on: January 8, 2015

Cellular pathogenesis in prion diseases.

Carole Crozet1, Florence Beranger, Sylvain Lehmann

  • 1Institut de Génétique Humaine, CNRS-UPR1142, 34396 Montpellier Cedex 5, France.

Veterinary Research
|April 17, 2008
PubMed
Summary

Prion diseases involve brain damage, including neuronal loss and abnormal prion protein accumulation. This review details how the prion protein (PrP) affects neuronal function, death, and the brain

Area of Science:

  • Neuroscience
  • Cellular Biology
  • Pathology

Background:

  • Prion diseases are neurodegenerative disorders characterized by specific pathological hallmarks.
  • These hallmarks include neuronal loss, spongiosis, astrocytosis, and microgliosis.
  • The accumulation of abnormal prion protein (PrPSc) in the central nervous system is a key feature.

Purpose of the Study:

  • To review the cellular pathogenesis of prion diseases.
  • To elucidate the mechanisms by which the prion protein (PrPC/PrPSc) impacts neurons.
  • To examine the relationship between PrPSc accumulation and neuronal cell death, and the role of PrPSc in glial reactions.

Main Methods:

  • This study is a review of existing literature on prion disease pathogenesis.
  • It focuses on analyzing the molecular and cellular mechanisms involved.

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

Published on: November 7, 2012

Related Experiment Videos

Last Updated: Jul 5, 2026

Investigating the Spreading and Toxicity of Prion-like Proteins Using the Metazoan Model Organism C. elegans
12:57

Investigating the Spreading and Toxicity of Prion-like Proteins Using the Metazoan Model Organism C. elegans

Published on: January 8, 2015

Monitoring Cell-to-cell Transmission of Prion-like Protein Aggregates in Drosophila Melanogaster
10:26

Monitoring Cell-to-cell Transmission of Prion-like Protein Aggregates in Drosophila Melanogaster

Published on: March 12, 2018

Protein Misfolding Cyclic Amplification of Prions
10:12

Protein Misfolding Cyclic Amplification of Prions

Published on: November 7, 2012

  • Key areas examined include neuronal dysfunction, cell death pathways, and neuroinflammation.

    • Main Results:

    • Prion protein isoforms (PrPC and PrPSc) play a critical role in neuronal dysfunction and death.
    • PrPSc accumulation correlates with the severity of neuronal loss and disease progression.
    • PrPSc triggers reactive astrocytosis and microgliosis, indicating a neuroinflammatory response.

    Conclusions:

    • The cellular pathogenesis of prion diseases is complex, involving direct effects of PrPSc on neurons.
    • Neuronal dysfunction and cell death are central events mediated by prion protein interactions.
    • PrPSc also modulates glial responses, contributing to the overall disease pathology.