Involvement of the immunological system in the pathogenesis of transmissible spongiform encephalopathies

Brun Torres, Alejandro; Castilla, Joaquín; Parra, B.; Rodríguez, Fernando; Torres, J. M.; Castilla, Joaquín [0000-0002-2216-1361]

Involvement of the immunological system in the pathogenesis of transmissible spongiform encephalopathies

2003

Objective. We review the current knowledge relative to the role of different immune system components and their contribution to the spread of prions throughout the infected organism. Development. During the last years research made on transmissible spongiform encephalopathies conducted to the assumption that changes in the structure of a determined protein (prion protein, PrP) may become self-propagative. The generally assumed finding of an abnormally folded protein as the causative agent, able to propagate in hosts different from it was originated, is changing our views of transmissible diseases. The concept of infection resulting from pathogen propagation within a susceptible host has been steadily linked to the ability of nucleic acids to replicate their own coded information. In the prionosis, term comprising all known prion disorders, the information seems to be coded within the own protein conformation. This phenomena, once again, challenges the well-established principles of molecular biology. Conclusions. Propagation of prions uses strategies not previously recognized, and depends upon prion conformation and/ or specific host restrictions. Thus, certain sheep and goat prion strains (scrapie) or human (variant of Creutzfeldt-Jakob disease) invade the brain after a initial interaction with the immune system of affected individuals. The involvement of the lymphoreticular system or, at least, certain host's immune competence seems to be a prerequisite for neuro-invasion after a natural infection or peripheral inoculation. On the other hand, proper identification of immune cell types involved may open the possibility for post exposure prophylaxis.

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