Particular attention in this field has been devoted to Prion proteins (PrP) due to their fundamental role as infective agents in diseases generally known as Transmissible Spongiform Encephalopathies (TSE) that affect humans and animals, including Creuzfeld-Jakob disease, fatal familial insomnia and Gerstmann-Straussler-Scheinker disease in humans, scrapie in sheep and mad-cow disease in cattle. The gene discussed is PRNP; the disease is Gerstmann-Straussler-Scheinker syndrome.