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Prion Diseases.

Benjamin C Whitechurch1,2, Jeremy M Welton1,2, Steven J Collins3,4

  • 1Department of Pathology, The University of Melbourne, Parkville, VIC, 3010, Australia.

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|July 5, 2017
PubMed
Summary
This summary is machine-generated.

Prion diseases are fatal neurodegenerative disorders caused by misfolded prion proteins. These infectious agents propagate misfolding, leading to varied clinical symptoms in humans and animals.

Keywords:
Creutzfeldt-Jakob diseasePrPCPrScPrion diseasePrion proteinTransmissible spongiform encephalopathy

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Area of Science:

  • Neurodegenerative diseases
  • Protein misfolding disorders
  • Transmissible spongiform encephalopathies

Background:

  • Prion diseases are fatal, transmissible neurodegenerative disorders.
  • They involve the misfolding of the normal cellular prion protein into infectious prions.
  • Prions propagate misfolding within and between cells, causing disease.

Purpose of the Study:

  • To describe the clinical manifestations of prion diseases.
  • To present evidence for the transmission of prion diseases by infectious proteins.
  • To explain the mechanism by which prion protein misfolding causes disease.

Main Methods:

  • Review of clinical phenotypes in humans and animals.
  • Analysis of prion strain conformations.
  • Examination of prion propagation mechanisms.

Main Results:

  • Prion diseases exhibit diverse clinical presentations.
  • Prion strains are determined by different misfolded protein conformations.
  • Misfolded prion protein acts as an infectious unit.

Conclusions:

  • Prion diseases are characterized by the propagation of misfolded prion proteins.
  • Prion strains contribute to the varied clinical phenotypes observed.
  • Understanding prion propagation is crucial for disease management.