Investigation of prion diseases.

Abstract

The prion diseases, or transmissible spongiform encephalopathies, are a group of fatal neurodegenerative disorders which occur in animals and man [4]. The first of these to be identified was the disease in sheep known as scrapie, which is endemic in many parts of the world including Europe and the USA. Scrapie has been extensively studied in experimental models to investigate the nature of the unique transmissible agents responsible for this group of disorders. Early studies revealed that the scrapie agent had a unique spectrum of physical and chemical properties, Table 1 Human prion diseasesFull size table with a marked resistance to conventional forms of microbial inactivation. In 1982, Prusiner published the prion hypothesis [62], which states that the transmissible agent responsible for this group of disorders is composed of a modified host protein, prion protein, which in humans is encoded by a gene on chromosome 20. Accumulation of the abnormal isoform of the prion protein (PrPres) in the brain is a characteristic feature of all prion diseases, and the detection of this abnormal protein is now of major importance in their diagnosis [40, 58, 59].KeywordsPrion ProteinPrion DiseaseBovine Spongiform EncephalopathyFatal Familial InsomniaHuman Prion DiseaseThese keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.