FIGURE 5.
Expression levels of PrPC in Tg(HuPrP) and Tg(NN6)h mice and sPMCA of sCJD MM1 + 2 PrPSc with human PrPC substrate. A, Western blot pattern and levels of PrPC and nonglycosylated PrPC(N181Q/N197Q) in the brain homogenate of Tg(HuPrP) and Tg(NN6)h mice, respectively, developed with mAb 12B2. B, relative expression levels of PrPC and PrPC(N181Q/N197Q) in Tg(HuPrP) and in Tg(NN6)h mouse brains, respectively, were measured with europium-labeled mAb 12B2 by CDI and compared with the levels in FVB mice. C, preferential amplification of type 1 PrPSc in sPMCA of mixed type 1 + 2 cases using Tg(HuPrP) and followed by WBs. The sPMCA was performed with homologous human PrPC as described previously (15) and 10-fold dilution of type 1 + 2 sCJD brain homogenate. The reaction product was after sPMCA and PK treatment deglycosylated with peptide:N-glycosidase F (PNGase F).