NOBELFÖRSAMLINGEN KAROLINSKA INSTITUTET THE NOBEL ASSEMBLY AT THE KAROLINSKA INSTITUTE 6 October 1997 has today decided to award the Nobel Prize in Physiology or Medicine for 1997 to Stanley B. Prusiner for his discovery of “Prions – a new biological principle of infection”. Summary The 1997 Nobel Prize in Physiology or Medicine is awarded to…

2. When a cow is fed with offals derived from a PrPSc-infected sheep, prions are somehow taken up from the gut and transported to the brain. The details of this process are not yet known, but one likely scenario is that PrPSc enters a nerve ending (synapse) from where it is transported along the nerve…

more

more

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.