Figure 6

Schematic representation of the deposition of white matter filamentous PrP in relation to the mutation: the horizontal bar represents the PRNP open reading frame with the N terminus on the left and the C terminus on the right. OPRI and point mutations are indicated with red symbols. At each mutation, the cases with white matter threads (filaments) are indicated with stars; the number of stars indicates the abundance of deposits (corresponding to the score in Table 2). For cases where no filaments are seen, white matter dot-like deposits are indicated by filled circles and cases with no white matter deposits are symbolised by open circles. The blue number next to the symbols corresponds to the case numbers in Table 2, all figures and in the text). The position of the mutations on the schematic representation is not exactly to scale.