DMMC Course MODEL SYSTEMS IN BIOMEDICAL RESEARCH: SCIENTIFIC BREAKTHROUGHS

1000-1115 Thursday 11 May 2006. UCD Conway Institute Seminar Room 2

Mouse: transgenic and knockout mice in prion research
Technical Spotlight: transgenic mouse production
Dr Mike Scott (UCD Conway Institute of Biomolecular and Biomedical Research)

The study of the prion protein (PrP) and the subsequent cloning of the PrP gene ushered in a new era in the study of the transmissible spongiform encephalopathies. The development of transgenic mice expressing foreign transgenes provided the first evidence that PrP was a major determinant of susceptibility to prions derived from other species. Gene-knockout mice with a targeted disruption of the PrP gene showed that organisms that lack PrP are completely resistant to infection by prions, and provided the first genetic proof that PrP was an essential component of the prion. PrP-knockin mice have also been created with precise substitutions of PrP sequences in their normal murine genomic context. These model systems have facilitated the development of exquisitely sensitive assays for prions, and provide excellent animal models for development of therapeutics for these invariably fatal conditions.