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Báo cáo khoa học: The spread of prions through the body in naturally acquired transmissible spongiform encephalopathies

Transmissible spongiform encephalopathies are fatal neurodegenerative dis-eases that are caused by unconventional pathogens and affect the central nervous system of animals and humans. Several different forms of these dis-eases result from natural infection (i.e. exposure to transmissible spongiform encephalopathy agents or prions, present in the natural environment of the respective host). | MINIREVIEW The spread of prions through the body in naturally acquired transmissible spongiform encephalopathies Michael Beekes1 and Patricia A. McBride2 1 Robert Koch-Institut P24 - Transmissible Spongiforme Enzephalopathien Berlin Germany 2 The Neuropathogenesis Unit Institute for AnimalHealth Edinburgh UK Keywords naturally acquired TSEs prion prion diseases prion protein prion routing prion spread transmissible spongiform encephalopathies Correspondence M. Beekes P24 - Transmissible Spongiforme Enzephalopathien Robert Koch-Institut Nordufer 20 13353 Berlin Germany Fax 49 30 4547 2267 Tel. 49 30 4547 2396 E-mail BeekesM@rki.de P. A. McBride 12 Gracemount Road Edinburgh EH16 6PH UK Fax Tel 44 131667 5204 E-mail tricia.mcbride@dsl.pipex.com Received 2 August 2006 revised 30 November 2006 accepted 4 December 2006 doi 10.1111 j.1742-4658.2007.05631.x Transmissible spongiform encephalopathies are fatal neurodegenerative diseases that are caused by unconventional pathogens and affect the central nervous system of animals and humans. Several different forms of these diseases result from natural infection i.e. exposure to transmissible spongiform encephalopathy agents or prions present in the natural environment of the respective host . This holds true also for scrapie in sheep bovine spongiform encephalopathy in cattle chronic wasting disease in elk and deer or variant Creutzfeldt-Jakob disease in humans all of which are assumed to originate predominantly from peroral prion infection. This article intends to provide an overview of the current state of knowledge on the spread of scrapie chronic wasting disease bovine spongiform encephalopathy and variant Creutzfeldt-Jakob disease agents through the body in naturally affected hosts and in model animals experimentally challenged via the alimentary tract. Special attention is given to the tissue components and spreading pathways involved in the key stages of prion routing through the body such as intestinal uptake .