[Prion diseases. Subacute spongiform encephalopathies in humans and animals (neural "anthropo- and zooprionoses")].
General review concerning current problems of subacute spongiform encephalopathies (Prion-diseases) in humans and in animals. The work is based on the aetiological conception of "Prion" as an allosteric conformational variant of the Prion-protein with autocatalytic "infectious" properties. The therm "Anthropoprionosis" and "Zooprionosis" are recommended by the author for human and animal Prion-diseases. Actual medical problems of various Prion-diseases are discussed in more detail, particularly those of aetiopathogenesis, genetics and molecular-biological principles in Prion-replication as well as the consensual neuropathological and clinical diagnostical criteria. Neuropathological findings are evaluated based on personal experience of diagnosed and/or consulted CJD-cases. On the ultrastructural level the morphological proof of abnormal secondary phagosomes in neurons of the CNS with accumulation of protein material ("prionosomes") is documented. Finally, the risks of CJD and the infectious potential of various organs and contaminated materials are also mentioned. In the methodical part, safety precautions in handling histological material from stereotactic biopsies and necropsies of brain are described, including safety techniques of autopsy in cases of CJD.
Ustav patologie, Palackého univerzita v Olomouci, Lékarská fakulta. 068/563@tunw.upol.cz
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D000818:Animals; D001921:Brain / Q000473:pathology; D002417:Cattle; D002418:Cattle Diseases / Q000175:diagnosis / Q000473:pathology / Q000635:transmission; D007562:Creutzfeldt-Jakob Syndrome / Q000175:diagnosis / Q000635:transmission / Q000821:virology; D016643:Encephalopathy, Bovine Spongiform / Q000175:diagnosis / Q000473:pathology / Q000635:transmission; D006801:Humans; D017096:Prion Diseases / Q000175:diagnosis / Q000235:genetics / Q000473:pathology / Q000635:transmission; D011328:Prions
D011328:Prions