Figure 1. PrP^180Q/196Q traffics similarly to WT PrP^C in primary neurons and in mice.

(A) Representative Western blot of PNGase-F–treated brain extracts from age-matched Prnp^180Q/196Q and WT mice reveal similar PrP^C expression levels (quantified in right panel) (100–250 day old mice); n = 4/group. (B) PrP immunocytochemistry shows that unglycosylated PrP^180Q/196Q traffics to neuronal processes in primary cortical neurons, as does PrP in WT neurons; n = 3 experiments. Scale bars: 10 μm. (C) Representative Western blots of phospholipase C–cleaved (PIPLC-cleaved) PrP^180Q/196Q and WT PrP from the surface of cortical neurons show that surface PrP^C levels are similar (media); n = 3 experiments. The additional band in the media (~23 kDa) may be a cleaved form of PrP. (D) PrP^180Q/196Q and WT PrP^C, together with flotillin, localize to detergent-resistant membranes in the brain; n = 3/group. Unpaired, 2-tailed Student’s t test, no significant differences (A and C).