Prion typing. Determination of electrophoretic mobility and PrPSc glycoform ratio in gPrD with E200G and E200K mutations. (a) Western blot analysis of PrPSc from an E200K-129V patient (lanes 1–3), our E200G case (lanes 4–6), an E200K-129M patient associated with PrPSc type 1 (lanes 9–11) and an E200K-129M patient associated with PrPSc type 2 (lanes 12–13). The mobility of the unglycosylated PrPSc from E200G is ~ 19 kDa as that of PrPSc type 2 and matches the corresponding mobility observed in E200K-129V and E200K-129M associated with PrPSc type 2. A fragment of ~17 kDa (indicated by the asterisk) was detected in the cerebellum of the two E200K-129V. PrPSc from sCJDMM1 (lane 7) and sCJDVV2 (lane 8) subtypes have been loaded as control. Frontal (lanes 1, 4, 7–9 and 12), parietal (lane 5), occipital (lanes 2, 10 and 13) cortices and cerebellum (lanes 3, 6 and 11) were examined. (b) The amounts of di-, mono- and unglycosylated PrPSc glycoforms expressed as percentage of the total PrPSc are virtually identical in all cases associated with the E200G and E200K mutations, but they differ from those of sCJDMM1 and sCJDVV2. Bar graphs are expressed as mean ± SEM of the two cortical regions from cases of E200K-129M (n = 3), E200K-129V (n = 4), and E200G, and one region from sCJDMM1 (n = 1) and sCJDVV2 (n = 1).