[Prion diseases and the biosecurity problems.]

The review presents the current state of the problem of prions and prion diseases with an emphasis on theepidemiological and epizootological risks of pathogens that cause fatal neurodegenerative diseases in humans and animals. The results of molecular genetic studies of the conversion of normal PrP^c prion protein molecules to infectious forms of PrP^d, resistance to physical disinfection methods, in particular exceptional thermal stability, and their ability to overcome interspecific barriers, while increasing virulence, are described. The possibility of infection not only by nutrition, when eating even heat-treated meat of sick animals, but also due to surgical interventions, especially neurosurgical and ophthalmic, as well as the use of immunobiological preparations, are emphasized. Since there are currently no means for the effective treatment of prion diseases in the world, attention is drawn to the high degree of relevance for the biosafety of the country to develop domestic highly sensitive test systems that can effectively detect prion infectious protein in vivo at the preclinical stage of the disease. The latest methods of automatic protein amplification and identification of prion proteins are briefly described as the most promising areas of research in the field of diagnosis of prion diseases.