MOLECULAR GENETICS OF TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES: AN INTRODUCTION

Abstract

Prnp knockout mice disrupted PrP^C-related genes have played an essential role to elucidate the relationship between PrP^C, a normal host gene product, and PrP^Sc, a protease-resistant, infectious PrP; Prnp knockout mice developed by Büeler et al. (1992) were completely protected against scrapie disease when challenged with mouse prions. Furthere, varying expression levels in PrP^C were revisited along with a varying susceptibility of mouse prions, when mouse Prnp genes were introduced into Prnp^o/o mice. How these murine models for human prion-related disease would contribute to the presently ongoing TSE research?