The aims of this study were: a) To describe the peripheral distribution of PrPsc in Spanish scrapie-affected goats; b) To differentiate, through immunohistochemical and biochemical methodologies, the strains causing the different scrapie cases (classical and atypical scrapie and BSE); c) To determine the PRNP genotypes of scrapie affected animals and healthy herd mates from four scrapie-affected flocks in a case-control study; and d) To compare these results with the PRNP variants observed in healthy animals from breed survey. This evidence concerns the gene PRNP and scrapie.