title: Transgenic mouse brains for the evaluation and quality control of BSE tests.
abstract: Rapid BSE tests are widely used diagnostics in veterinary medicine and more than 11 million tests are applied worldwide. The evaluation of new rapid BSE tests and the quality assurance of approved BSE tests pose a challenge owing to the natural scarcity of BSE-infected bovine brainstems and regional variations in prion titer. Transgenic mice expressing bovine prion protein (Tg4092) offer an alternative approach to these problems. To determine whether BSE-infected Tg4092 mouse brains could serve as a general standard for rapid BSE tests, we inoculated Tg4092 mice intracerebrally with BSE prions, harvested brains at defined time points post-infection and analyzed cerebral hemispheres with several approved rapid BSE tests. The results show that de novo formation of the disease-causing prion protein isoform, PrP(Sc), can be monitored during the course of infection. We demonstrate that BSE-infected Tg4092 mouse brains provide a renewable and controllable source of reference samples and suggest that such samples can generally be used for the evaluation and quality control of rapid BSE tests.
authors.affiliation: European Commission, Directorate General Joint Research Centre, Institute for Reference Materials and Measurements, B-2440 Geel, Belgium. wolfgang.philipp@ec.europa.eu
authors.forename: Wolfgang J
authors.initials: WJ
authors.lastname: Philipp
authors.forename: Darlene
authors.initials: D
authors.lastname: Groth
authors.forename: Kurt
authors.initials: K
authors.lastname: Giles
authors.forename: Pavel
authors.initials: P
authors.lastname: Vodrazka
authors.forename: Heinz
authors.initials: H
authors.lastname: Schimmel
authors.forename: Muriel
authors.initials: M
authors.lastname: Feyssaguet
authors.forename: Reet
authors.initials: R
authors.lastname: Toomik
authors.forename: Pascal
authors.initials: P
authors.lastname: Schacher
authors.forename: Awad A
authors.initials: AA
authors.lastname: Osman
authors.forename: Ingolf
authors.initials: I
authors.lastname: Lachmann
authors.forename: Angus
authors.initials: A
authors.lastname: Wear
authors.forename: Jean-Noel
authors.initials: JN
authors.lastname: Arsac
authors.forename: Stanley B
authors.initials: SB
authors.lastname: Prusiner
mesh_terms: D000818:Animals; D002417:Cattle; D004195:Disease Models, Animal; D016643:Encephalopathy, Bovine Spongiform / Q000378:metabolism / Q000503:physiopathology*; D051379:Mice; D008822:Mice, Transgenic; D018620:PrPSc Proteins / Q000378:metabolism*; D011328:Prions / Q000235:genetics*; D011786:Quality Control
chemical_list: D018620:PrPSc Proteins; D011328:Prions