Transmissible spongiform encephalophaties: common characteristic, theories of the development, biological models of the investigation

M. R. Kozak, R. S. Stoika, Yu. Ya. Kit, V.V. Vlizlo

For today a question of classification and definition of prion diseases of man and animals in the article of discussion among the specialists of different type, which engage in this problem. Prion diseases are divided into infectious, genetically conditioned and sporadic cases. The pathogens which cause transmissible spongiform encephalophaties are prion proteines with the changed tertiary structure. The inherited cases of prion diseases are the consequence of mutation of gene which encodes a prion protein structure. Sporadic transmissible spongiform encephalophaties happen, when a patient in no way contacted with pathogenic prions and his genome does not contain a mutation in the gene of PRNP. Yeasts prion proteins forming amyloid fibrils at presence of the mutations in a gene, which encode the structure of these proteines, however pathological process not development. If not all prion diseases, the reason of origin of sporadic transmissible spongiform encephalophaties, possibly, is development of autoimmune disease.

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