This animation gives a very simplified view of how prions cause fatal neurodegenerative diseases of the brain in man and animals next step 1. The prion protein, PrP, is a normal constituent of many cell types in the body, notably nerve cells (neurons) in the brain. The name prion (pronounced pree-on) is derived from”proteinaceous infectious…

5. In neurons infected with the prion protein, more and more PrPSc will gradually be produced forming larger fibrillar aggregates. When a critical level of PrPSc has accumulated, the neurons die, resulting in large vacuoles. The affected brain regions become sponge-like in appearance, hence the name spongiform encephalopathies, to describe all prion diseases.  

4. In hereditary forms of prion diseases, a genetic change (a mutation represented by the red dot) in one of the two genes encoding PrPc, may result in a slightly altered structure of the PrPc-protein. The mutation makes it easier for PrPc to change its conformation into PrPSc, initating a chain reaction that will result…

An animation illustrating the discovery the 1997 Nobel Prize in Physiology or Medicine, shown during the press conference on October 6, 1997 at Karolinska Institutet, is presented here. The latter part of the presentation contains only images and text. This is a 2.7 Mb Shockwave animation. To download the Shockwave player, click on the icon…

8. In the 1950s and 1960s, the Nobel Laureate studied a disease called kuru which spread among the Fore people in New Guinea through cannibalistic rituals. Kuru means shivering or trembling in the Fore language. The picture, taken by Gajdusek in 1957, shows an 8-year old girl in an advanced stage of kuru.

6. This microscopic picture shows a histological section from a prion-infected brain. (1) Vacuoles that are formed as a result of neuronal cell death, results in a sponge-like appearance of the brain.  

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…