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Figure S1. Changes to the lesion scoring protocol allows UK-1 to be distinguished from bovine spongiform encephalopathy (BSE). Lesion profiles (LP) following transmission of UK-1, ovine and bovine BSE to RIII mice. The G1 scoring area was split so that the dorsal medulla nuclei (G1d) and the cochlear nuclei (G1c) were scored independently. The numbers of animals that contributed to each profile are given in parentheses. Error bars indicate standard error of the mean. Mice were all clinically and pathologically positive.

Figure S2. Retrospective analysis of ten classical scrapie sources using the alternative lesion scoring protocol. Lesion profiles (LP) following transmission of 10 classical scrapie sources (red lines) and 10 bovine spongiform encephalopathy (BSE) sources (black lines) to RIII mice as previously detailed (3). The dorsal medulla nuclei (G1d) and the cochlear nuclei (G1c) were scored independently. Error bars indicate standard error of the mean. Only mice with both dorsal medulla and cochlear nucleus present were included. Mice were all clinically and pathologically positive. Each profile was constructed from at least five mice.

Figure S3. UK-1 and UK-2 LP following transmission to MR and SJL/OlaHsd mouse lines. LP following transmission of UK-1 (A) and UK-2 (B) to MR mice and UK-2 to SJL/OlaHsd mice (C), are shown. Where less than five clinically and histopathologically positive mice were available, separate profiles representing all hematoxylin and eosin (H&E)-positive mice (dashed line) and mice that were both clinically and H&E-positive (solid line) are plottedalongside representative bovine spongiform encephalopathy (BSE) profiles. Error bars indicate standard error of the mean. The numbers of animals which contributed to each profile are given in parentheses. UK-1 did not transmit to SJL/OlaHsd mice.

Figure S4. PrPSc patterns associated with scrapie strains 87A, 87V and ME7. A. 87A in a C57BL mouse. B. 87V in a VM mouse. C. ME7 in an RIII mouse. Scale bars represent 500 μm.

Figure S5. Western blot analysis of UK-2 transmissions to tg338 mice and UK-1 and UK-2 transmissions to TgshpXI mice. A. Detection of PrPSc by Sha31 and P4 monoclonal antibodies in proteinase-K treated tg338 brain homogenates following challenge with UK-2. Lane 1: original ovine inoculum, lanes 2–11: inoculated tg338 mouse brains, lanes 12 and 15: bovine BSE control, lane 13: experimental bovine spongiform encephalopathy (BSE) in sheep control, lane 14: classical scrapie control. B. Detection of PrPSc by Sha31 and P4 monoclonal antibodies in proteinase-K treated TgShpXI brain homogenates following challenge with UK-1 and UK-2. Lanes 1–4 and 7–10: TgshpXI mice inoculated with UK-1 and UK-2, respectively. Lane 5: UK-1 challenged tg338 mouse, lane 6: blank, lane 11: UK-2 challenged tg338 mouse, lanes 12–13: unchallenged TgshpXI mouse −/+ proteinase-K, lane 14: bovine BSE, lane 15: ovine scrapie. Molecular mass markers (in kDa).

FilenameFormatSizeDescription
BPA_526_sm_FS1.pdf264KSupporting info item
BPA_526_sm_FS2.pdf286KSupporting info item
BPA_526_sm_FS3.pdf341KSupporting info item
BPA_526_sm_FS4.pdf19860KSupporting info item
BPA_526_sm_FS5.pdf2884KSupporting info item

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