Additional supporting information can be found in the online version of this article.

ANA_22104_sm_suppinfofig1.tif5123KSuppl. Fig. 1. Chimeric mouse/human transgene sequences. (A) Alignment of mouse and human PrP sequences, with N- and C-terminal signal peptides shaded, with differences in the mature sequence indicated by boxes. Asterisk indicates polymorphic residue in human PrP. (B) Schematic representation of mature mouse (green) and human (orange) PrP and mouse/human chimera MHu2M. Due to the high similarity of mouse and human PrP, the MHu2M chimera can also be represented as nine point mutations in the mouse PrP sequence. This is the construct in the Tg5378 mice. (C) Schematic representation of MHu2M(M165V,E167Q) and MHu2M(M111V,M165V,E167Q) constructs, from the Tg22372 and Tg1014 lines, respectively. HuPrP residues indicated by orange circles. (D) Reduction of incubation periods to human prions in successive lines of transgenic mice. Incubation periods (arrows) and susceptibility (percentages) for intracerebral inoculation sCJD (red) and vCJD (blue) prions in three transgenic mouse lines.
ANA_22104_sm_suppinfofig2.tif602KSuppl. Fig. 2. Strain typing of Tg mouse brains from primary transmission of vCJD prions by Western blotting after limited digestion with PK. (A) vCJD case RU96/02 (lane 1) and its passage Tg1014 mice (lanes 2–6), (B) vCJD case RU96/07 (lane 1) and its passage in Tg1014 mice (lanes 2–9). The incubation period for each mouse is listed above the respective lane. Molecular weight markers represent protein standards of 20 (top) and 30 (bottom) kilodaltons.
ANA_22104_sm_suppinfofig3.tif7547KSuppl. Fig. 3. Serial passage of vCJD prions in Tg(MHu2M)5378 mice: (A) RU96/07 isolate (B) RU96/02 isolate. Each box represents a single mouse, with its incubation period in days (ns, not sick) and PrPSc strain type indicated in color: type 1 (blue), type 2 (red), types 1 and 2 (purple), not determined (white). From center, inner ring shows the first passage with human vCJD brain homogenate, middle ring shows the second passage, and outer ring shows the third passage. For each serial passage, brain homogenate from the respective ill Tg5378 mice was prepared and inoculated into other Tg5378 mice.
ANA_22104_sm_suppinfotables.doc58KSuppl. Table 1. Serial transmission of vCJD prions to Tg lines expressing chimeric mouse/human PrP.* Suppl. Table 2. Incidence of strain type upon serial passage.*

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