,4 Critical to prion disease pathogenesis is the ability of PrPSc to recruit PrPC and through an incompletely understood templating mechanism involving binding to PrPC by which PrPC is induced to misfold and convert into PrPSc, thereby progressively leading to a toxic accumulation of PrPSc, neurodegeneration and death.5 The gene discussed is PRNP; the disease is prion disease.