EN
Prion diseases (for example: scrape of sheep, BSE, CJD of humans) are among the most notable central nervous system degenerative disorders caused by the accumulation of modified cellular protein. The conversion of PrP(C) (the normal cellular protein) into PrP(Sc) (the abnormal disease-causing isoform) involves a conformation change whereby the ?-helical content diminishes and the amount of ? sheet increases. PrP (Sc) is partially resistant to proteases, temperature, high and low pH. Because the incidence of prion diseases is due to several factors, various efforts need to be taken to reduce the scale and consequences of the disease. They include post-mortem and in vivo diagnosis and prophylactics, i.e. monitoring of animals and feed control.