The conversion of the cellular prion protein PrPC to a misfoldedβ-rich conformer called PrPSc underlies a group ofneurodegenerative diseases known as transmissible spongiform encephalopathies (TSEs).PrPSc is self-propagating, i.e, capable of inducing the conversion ofnaïve PrPC molecules to the misfolded conformation1 and the accumulation of sufficient levels of PrPSc resultsin the formation of oligomers and higher-order fibrillar aggregates. Here, PRNP is linked to glycogen storage disease VI.