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

Published on: January 8, 2015

Prion protein functions and dysfunction in prion diseases

Akikazu Sakudo1, Kazuyoshi Ikuta

  • 1Department of Virology, Research Institute for Microbial Diseases, Osaka University, Yamadaoka, Suita, Osaka 565-0871, Japan. sakudo@biken.osaka-u.ac.jp

Current Medicinal Chemistry
|January 20, 2009
PubMed

Insights

Prion diseases stem from misfolded prion proteins (PrPSc) damaging brain cells. This review covers prion protein (PrPC) roles and PrPSc involvement in disease development and neuronal death.

Area of Science:

  • Neuroscience
  • Infectious Diseases
  • Biochemistry

Background:

  • Prion diseases are zoonotic disorders caused by infectious particles known as prions.
  • The primary component of prions is a misfolded, partially protease-resistant conformer (PrPSc) of the normal cellular prion protein (PrPC).
  • PrPC's anti-oxidative function is suggested by studies using PrP-knockout models.

Purpose of the Study:

  • To review current knowledge on cellular prion protein (PrPC) and misfolded prion protein (PrPSc).
  • To elucidate the involvement of PrPC and PrPSc in the pathogenesis of prion diseases.
  • To summarize the key features and transmission routes of prion diseases.

Main Methods:

  • Literature review of existing research on prion diseases.
  • Analysis of studies on PrPC function and PrPSc formation.
  • Synthesis of information regarding prion disease pathogenesis.

Main Results:

  • Prion diseases are characterized by PrPSc deposition, astrocytosis, and vacuolation, influenced by prion strain and host species.
  • Prions initially replicate in the lymphoreticular system before neuroinvasion via peripheral nerves.
  • Prion conversion leads to PrPSc accumulation and PrPC deficiency, causing neuronal death through apoptosis and autophagy.

Conclusions:

  • Understanding the roles of PrPC and PrPSc is crucial for comprehending prion disease pathogenesis.
  • The interplay between prion strains, host factors, and disease progression requires further investigation.
  • Further research into the mechanisms of neuronal damage, including apoptosis and autophagy, is essential.

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