This animation gives a very simplified view of how prions cause fatal neurodegenerative diseases of the brain in man and animals
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 particle”. In its normal conformation, the prion protein (termed PrPc) is innocuos. Its function in the body is still unknown. However, PrPc can undergo a conformational change, adopting a harmful, disease-causing shape, termed PrP-scrapie, or PrPSc. Scrapie is a transmissible spongioform encephalopathy of sheep identified in Iceland already in the l8th century.
The conversion of the PrPc-form to the PrPSc-form occurs at an extremely low frequency. The process may be compared to the transformation of Robert Louis Stevenson’s novel character Dr Jekyll to Mr Hyde – the same entity, but in two manifestations, a “kind” innocuous one, and a “vicious”, lethal one. PrPSc has unusual properties. It is stable to treatments at high temperatures ( 100°C), UV-light, and ionizing irradiation, enzymes that normally destroy proteins and nucleic acids, and some detergents. Such treatments would destroy all known viruses.
Their work and discoveries range from how cells adapt to changes in levels of oxygen to our ability to fight global poverty.
See them all presented here.