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…

  Presentation Speech by Professor Ralf F. Pettersson of the Nobel Committee at , December 10, 1997. Translation of the Swedish text. Your Majesties, Your Royal Highnesses, Ladies and Gentlemen, This year’s Nobel Prize in Physiology or Medicine has been awarded to Stanley B. Prusiner for his discovery of prions – a new biological principle…

Scientific Advisors: Professors at the Karolinska Institute Krister Kristensson, Neuropathology, Ralf Pettersson, Molecular Biology Bengt Winblad, Geriatrics, Nils Ringertz, Medical Cell Genetics and Secretary of the Nobel Assembly. References: Stanley B Prusiner, The Prion Diseases, Scientific American 272, 30-37 (1995) A.Aguzzi, Prion research: the next frontier. Nature 389, 795-798 (1997). K Kristensson and B. Winblad,…

 PrPc   The prion protein exists in two forms. The normal, innocuous protein (PrPc) can change its shape to a harmful, disease-causing form (PrPSc). The conversion from PrPc to PrPSc then proceeds via a chain-reaction. When enough PrPSc proteins have been made they form long filamentous aggregates that gradually damage neuronal tissue. The harmful PrPSc…

My history is not atypical of many Americans: born in the midwest, educated in the East, and now living in the West. My early years were shared between Des Moines, Iowa and Cincinnati, Ohio. Shortly after I was born on May 28, 1942 in Des Moines, my father, Lawrence, was drafted into the United States…

Prions are simple proteins that are much smaller than viruses. They are unique since they lack a genome. All other known infectious agents contain genetic material. Bacteria are often surrounded by a strong protective cell wall and replicate through simple cell division. Fungi may cause infections of the mouth, throat, lungs, and skin. Parasites thrive…

Contents: These pages are based on material from the 1997 Physiology or Medicine Nobel Poster.

  The Nobel Assembly at the Karolinska Institute in Stockholm, Sweden, has awarded the Nobel Prize in Physiology or Medicine for 1997 to Stanley B. Prusiner, for his discovery of “prions – a new biological principle of infection”. Stanley B. Prusiner was born in 1942 in Des Moines, Iowa, USA. Since 1968 he has been…

Prions affect different regions of the brain. A sponge-like appearance results when nerve cells die. Symptoms depend on which region of the brain is affected. Cerebral cortex When the cerebral cortex is affected, the symptoms include loss of memory and mental acuity, and sometimes also visual imparement (CJD). Thalamus Damage to the thalamus may result…

1. Through horizontal transmission from e.g. a sheep to a cow (BSE). 2. In inherited forms, mutations in the prion gene are transmitted from parent to child. 3. They can arise spontaneously. Route of infection When cows are fed with offals prepared from infected sheep, prions are taken up from the gut and transported along…