Figure 1

Comparison of KA-induced seizure profile in Prnp^+/+and Prnp^0/0 mice on B6129, B6.129, 129/Ola or FVB/N genetic backgrounds.

(a) Percentage of mice reaching seizure stages I to VI and KA-induced mortality. Adult animals were subjected to a multiple KA-injection protocol (10 mg/kg b.w.) and epileptic responses were analysed during 4 hours after the first KA injection (see Methods for details).(b) Example of the western blot detection of PrP^C (6H4) expression in brain extracts obtained from B6129 Prnp^Zrchl/Zrchl Prnp^+/+, B6129 Prnp^Zrchl/Zrchl Prnp^0/0, 129/Ola Prnp^Edbg/Edbg Prnp^+/+, 129/Ola Prnp^Edbg/Edbg Prnp^0/0, FVB/N Prnp^+/+ and FVB/N Prnp^0/0 mice. Tubulin was used as a loading control. (c) Photomicrographs showing the pattern of neurodegeneration (Fluoro-Jade B staining) and DAPI staining of hippocampus in B6129 Prnp^Zrchl/Zrchl Prnp^+/+, B6129 Prnp^Zrchl/Zrchl Prnp^0/0, 129/Ola Prnp^Edbg/Edbg Prnp^+/+, 129/Ola Prnp^Edbg/Edbg Prnp^0/0, 24 hours after KA treatment. Dying cells are mainly located in the pyramidal cell layer of CA1 and CA3 regions of B6129 Prnp^Zrchl/Zrchl Prnp^0/0 and 129/Ola Prnp^Edbg/Edbg Prnp^0/0 mice respectively. (d) Time course of stage VI seizure during the first 30 min, from 30–60 min and from 60–180 minutes, of adult B6129 Prnp^Zrchl/Zrchl Prnp^0/0 vs B6.129 Prnp^Zrchl/Zrchl Prnp^0/0. Data are presented as the mean ± S.E.M. of the number of seizures/number of animals. Note the lower levels of seizures in parallel with decreased 129 associated genes in B6.129 Prnp^Zrchl/Zrchl Prnp^0/0 mice. Abbreviations: sr, stratum radiatum; sl, stratum lucidum; slm, stratum lacunosum-moleculare; sp, stratum pyramidale; so, stratum oriens. Scale bar represents 200 μm. Asterisks indicate statistical significance (***P < 0.01, Mann-Whitney U test).