Modelos animales para la investigación de priones

Abstract

Spongiform encefalopathies are a group of diseases with very long incubation times, during which the pathogenic isoform PrPres accumulates slowly in the brain, till it reaches a high enough concentration as to develop clinical signs. To study these diseases, and especially to determine the etiological relationships between the PrPres from different diseases, animal models are used, mostly transgenic mice, in which their own Prnp gene is substituted by that from other species. In this way, the lesions and vacuolization pattern, as well as the Western Blot profile are like those in the original species, with the advantage that the course of the disease is faster and the convenience of infrastructures and ethical considerations of working with mice.