PrP^CGPIThy-1 L150 mice show a delay to terminal disease and altered neuropathology upon prion infection.

(A) Representative blot of PrPGPIThy-1 L150 (generated for infection experiments) and WTPrPC mouse brain homogenates showing no relevant differences in the amount of PrPCGPIThy-1 and WTPrPC in the brain. Chart underneath shows mean relative intensity of PrP referred to actin where WTPrPC samples are set to 100% as a reference (error bars are SEM; n = 5 for each genotype). (B) Kaplan-Meier survival curve. Mice were inoculated intracerebrally (i.c.) with RML prions and sacrificed at terminal disease. Note the significant delay (****pCGPIThy-1 L150 (n = 10) and WTPrPC mice (n = 8) incubation time. (C) Neuropathological characterization of terminally diseased mice. Note that PrPCGPIThy-1 L150 mouse brains show a general decrease in spongiosis as observed with HE staining. Gliosis is also decreased in the transgenic mice as manifested by reduced GFAP (astrocytes) and Iba1 staining (microglia) in all the studied regions. (D) Lesion profile after semiquantitative assessment (n = 3 or n = 4 for each genotype) of spongiosis and gliosis (FC: frontal cortex; H: hippocampus; S: striatum; P: pons; Cb: cerebellum). Note the general decrease in lesion severity for the PrPCGPIThy-1 L150 mice (red line), compared to WTPrPC profile (green line).