CHARACTERIZATION OF CLASSICAL AND ATYPICAL BSE IN GOAT:CLINICAL SIGNS, PATHOGENESIS AND DIAGNOSTIC ELEMENTS

The identification and characterization of atypical BSE is certainly one of the most interesting study in prion diseases. The identification, for the first time in Italy, of an atypical form of BSE in cattle called BASE (Bovine Amyloidotic Spongiform Encephalopathy) has given the way to a new line of comparative research of new forms of TSEs. Preliminary results of these studies have shown more aggressiveness of BASE than classical BSE, that is able to infect all species in which the disease was transmitted, including non-human primates and transgenic mice. Furthermore, BASE has shown a remarkable plasticity, being able to modify its pathological and biochemical phenotype depending on the species in which it was transmitted. In this regard, some of the results obtained in wild type mice let hypothesize a possible new theory about the origin of BSE. In SJL and C57BL mice in fact, BASE showed the ability to convert into BSE. Therefore it is possible that BSE agent could originate from BASE strain, after a conversion in peripheral tissues or in an intermediate host. Hence the need for a study of experimental transmission in goat species in which up to now the BASE has not been transmitted, in order to characterize this species in clinical pathological and biochemical BASE phenotype. The study allowed us to confirm the susceptibility of goats to atypical BSE, characterizing the phenotype from a pathological point of view. In addition, goat is a species of great interest of public health due to the fact that it is already susceptible to classical BSE. Therefore the study, through the application of highly sensitive diagnostic techniques for prion protein in central nervous system and in peripheral tissues will determine whether there are possible risks to humans if BASE, as BSE, could infect caprine species.