Fig. 4.

Diagrams of brain, spinal cord, and retina neuropathology in polyQ models that use the PrP promoters. As in previous figures, the phenotypes and brain regions were marked with colors on a schematic sagittal section of the mouse brain. Because multiple PrP polyQ models were available for some diseases, the data from multiple models for each disease were pooled to increase the total information content. The PrP promoter produces a pattern of neuropathology that is different from that produced in models that use native promoters. The PrP promoter tends to produce neuropathological outcomes in the cerebral cortex (CTX), cerebellum (CB), and striatum (STR), but, unlike the native promoters (Fig. 3), the PrP promoter does not tend to produce pathology in the hippocampus (HP)