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Prions and the prion disorders

E Fisher1, G Telling, J Collinge

  • 1Department of Neurogenetics, Imperial College School of Medicine at St. Mary's, London, UK.

Mammalian Genome : Official Journal of the International Mammalian Genome Society
|July 10, 1998
PubMed
Summary

Prion disorders, including scrapie and Creutzfeldt Jakob disease (CJD), are caused by infectious agents called prions. Research indicates these prions lack nucleic acid and consist of an abnormal prion protein (PrP) isoform.

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Area of Science:

  • Neuroscience
  • Infectious Diseases
  • Molecular Biology

Background:

  • Prion diseases, such as scrapie in sheep and Creutzfeldt Jakob disease (CJD) in humans, have long fascinated biologists due to their unique disease mechanisms.
  • These disorders gained significant attention with the bovine spongiform encephalopathy (BSE) epidemic and the emergence of a new variant CJD.

Purpose of the Study:

  • To review evidence concerning the nature of the infectious agent responsible for prion disorders.
  • To elucidate the composition of the prion, the causative agent of these neurodegenerative diseases.

Main Methods:

  • Review of existing scientific literature and studies on prion diseases.
  • Analysis of experimental data converging on the composition of the infectious agent.

Main Results:

  • Evidence strongly suggests the infectious agent, termed 'prion', is devoid of nucleic acid.
  • The prion is composed of an abnormal isoform of the host-encoded prion protein (PrP).

Conclusions:

  • The prion is a proteinaceous infectious particle, challenging traditional infectious agent paradigms.
  • Understanding the prion's structure is crucial for comprehending and potentially treating prion-related neurodegenerative diseases.

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