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Biology and Genetics - Prion Diseases

Prion diseases are a unique group of neurodegenerative disorders that can be transmitted among humans and animals, mainly sheep and cows.

Prion diseases or TSE’s, transmissible spongiform encephalopathies, affect the central nervous system causing the brain to develop sponge-like holes which destroy its efficacy. Even though these diseases have long incubation periods, often over ten years,    once the clinical symptoms appear, deterioration is continuous and deadly.  Unlike other fatal illnesses that are caused by bacteria, virus or parasites, prion diseases are unique:

  • Can be inherited;
  • Can occur sporadically;
  • Can be transmitted through different routes

The prion is the newest infectious agent, we know, a non-living protein.  Standard methods to dismantle its power like chlorination, ultra-violet radiation, or intense heat do not prevent transmission, nor is there a diagnostic approved yet. This is a challenging situation. Regulatory agencies that are responsible for both public and veterinary health must be cautious. The outbreak of BSE in livestock and vCJD in a small number of humans in recent years has spurred research to determine therapeutic interventions for TSE’s in the near future.

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Contributor: Shalu Darshan

Last Reviewed: June 2nd, 2010


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