Understanding Prion Diseases

Prion Diseases such as Kuru are a class of rare neurodegenerative disorders that affect both humans and animals. They are also known as transmissible spongiform encephalopathies. They are distinguished by long incubation periods (in the order of years to decades), characteristic spongiform changes associated with neuronal loss, and a failure to induce inflammatory response.

The causative agents of TSEs are believed to be prions. The term "prions" refers to abnormal, pathogenic agents that are transmissible and are able to induce abnormal folding of specific normal cellular proteins called prion proteins that are found most abundantly in the brain. The functions of these normal prion proteins are still not completely understood. The abnormal folding of the prion proteins leads to brain damage and the characteristic signs and symptoms of the disease. Prion diseases are usually rapidly progressive and always fatal.

The most commonly known TSE is Mad Cow Disease or Bovine Spongiform Encephalopathy (BSE).


One of the biggest misconceptions about Mad Cow Disease and Prion Diseases is that cooking makes the beef, or in the case of the fore the human, safe to eat. This is simply not true as the prion protein can survive extremely high temperatures, so cooking will not protect anyone from this abnormal prion protein.


Sources Referenced and Cited
http://www.cdc.gov/ncidod/dvrd/prions/
http://www.umm.edu/features/madcow.htm