Prions and prion diseases: myths and reality
E.YU. SKRIPCHENKO1, 2, K.V. MARKOVA1, N.V. SKRIPCHENKO1, 2, A.V. GOLUBEVA1
1Pediatric Research and Clinical Center for Infectious Diseases, Saint Petersburg
2Saint Petersburg State Pediatric Medical University, Saint Petersburg
Contact details:
Skripchenko E.Yu. — MD, Associate Professor, Head of the Research department of neuroinfections and organic pathology of the nervous system, Professor of the Department of neonatology with courses of neurology and obstetrics-gynecology
Address: 9 Prof. Popov St., Saint Petersburg, Russian Federation, 197022, tel.: +7 (812) 234-10-04, e-mail: wwave@yandex.ru
The article contains modern data about prions and the main prion-associated diseases. The authors describe the main conditions that provide prion-associated infectious diseases, primarily the conformation changes of protein PrPС expressed in healthy tissues of humans and animals and forming the pathogenic PrPSc protein. All prion-caused diseases are considered to be neurodegenerative with fatal outcomes due to a specific spongy brain damage. Information on some aspects of pathogenesis and therapeutic options for prion diseases is presented. The data on the prion safety of biological preparations widely used in healthcare are also presented.
Key words: prions, prion-associated diseases, pathogenic protein, therapy, safety of biological products.
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