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  Authority of Polish Society of Allergology

vol 3. no 2. June 1998  

 Clinical immunology
Still fascinating prions
Paweł P. Liberski

Samodzielna Pracownia Biologii Nowotworów Katedry Onkologii Akademii Medycznej, ul.Paderewskiego 4, 93-509 Łódź

Transmission of bovine spongiform encephalopathy (BSE) or "mad cow disease" to humans resulted in a new variant of Creutzfeldt-Jakob disease and raised both fear in media and a new wave of interest in prion diseases or transmissible spongiform encephalopathies. "Prions" (according to a widely accepted definition) are proteinaceus infectious particles composed of an abnormal conformer of prion protein (PrP). Indeed, scrapie and cellular isoforms of PrP are identical in amino acid sequence but different in conformation (a relative proportion of alpha-helices and beta-sheets). Prions are transmissible agents of kuru, Creutzfeldt-Jakob disease, scrapie and several other "transmissible spongiform encephalopathies". However, these agents may not only be prions but also viruses or virinos (a viroid-like nucleic acid embedded in a host-encoded protein shell).


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