Fig. 5
From: Homozygous R136S mutation in PRNP gene causes inherited early onset prion disease
In vitro human misfolded PrP propagation by recPMCA using R136 vs. S136 human as substrates. Human 129M 136R (wt) rec-PrP, human 129M 136S rec-PrP, and a mix of both rec-PrPs in ratio 1:1 were complemented with chicken brain homogenate, seeded with different dilutions of recPMCA-adapted CJD MM1 misfolded rec-PrP (10-1–10-8) and subjected to a unique 24 h round of standard recPMCA. Amplified samples were digested with 50 μg/ml of Proteinase-K and analyzed by Western blot using monoclonal antibody D18 (1:5000). No significant differences were found in the propagation ability of the R136 (wt) vs. the mutant R136S rec-PrP or the mix 1:1 of both proteins