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Bovine spongiform encephalopathy, scrapie and Creutzfeldt–Jakob disease are examples of prion diseases. These diseases are sometimes referred to as transmissible spongiform encephalopathies (TSEs). Prion diseases require the expression of a normal host protein, termed the prion protein, and its conversion to an abnormal isoform. The abnormal isoform of the prion protein that accumulates in disease is termed PrPSc and is tightly associated with the infectious agent of TSEs .