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The Nobel Prize in Physiology or Medicine 1997

Prions infect animals and humans
All known prion diseases are fatal. Since the immune system does not recognize prions as foreign, no natural protection develops. Scrapie in sheep was first described during the18th century. It has been transmitted to other animals such as mink and cats, and more recently to cows (mad cow disease or bovine spongiform encephalopathy, BSE) through contaminated feedstuff.
 

In New Guinea, the Fore-people contracted kuru by eating the brains of deceased people. Creutzfeldt-Jakob Disease (CJD) frequently arises spontaneously, while fatal familial insomnia (FFI) Gerstmann-Sträussler-Scheinker (GSS) disease, and 10-15% of CJD are caused by mutations in the gene encoding the prion protein. A new variant CJD, diagnosed in some 20 patients, may have arisen through transmission of BSE to humans.

 
Contents:
Introduction » Prions - novel infectious agents differing from all other known pathogenic agents » Prions infect animals and humans » From Dr Jekyll to Mr Hyde » Different prions affect different regions of the brain » Prion disease arise in three different ways »
These pages are based on material from the 1997 Physiology or Medicine Nobel Poster.
Credits and references for the poster »

 

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