Figure 4.

Summary of molecular and neuropathological phenotypes in transgenic mice. (a) Molecular strain typing of human prion transmissions to mice. Immunoblots of proteinase K-digested brain homogenates from human patients or transgenic mice analysed by enhanced chemiluminescence with anti-PrP monoclonal antibody 3F4. The provenance of each brain sample is designated above each lane and the type of human PrP^Sc detected in each sample (using the London classification of human PrP^Sc types; Collinge et al. 1996; Hill et al. 2003) is designated below. (b) Neuropathological analysis of transgenic mouse brain. An equivalent pattern of neuropathology is seen in 129VV Tg152 mice that propagate type 3 PrP^Sc following primary transmission of kuru prions or sporadic CJD prions, which is distinct from 129VV Tg152 mice that propagate type 5 PrP^Sc following primary transmission of vCJD prions. Sketches represent the regional distribution of abnormal PrP deposition in transgenic mouse brain visualized with anti-PrP monoclonal antibody ICSM 35; bars, diffuse synaptic PrP deposition and circles, PrP plaques.