Our results showed that PDK4 was upregulated in the presence of the natural Gerstmann-Sträussler-Scheinker syndrome (GSS) variant of PrP (A117V) that is known to generate CtmPrP in abundance and ER stress (Supplementary Fig. 2C, D). Here, PDK4 is linked to Gerstmann-Straussler-Scheinker syndrome.