prion protein disease

prion protein disease

One of a number of transmissible diseases, including CREUTZFELDT-JAKOB DISEASE, Gerstmann-Straussler syndrome and KURU, all of which feature a SPONGIFORM ENCEPHALOPATHY. They are all associated with an abnormal form of a normal cell protein called PRION PROTEIN. They feature plaques of AMYLOID in the brain tissue and the prion protein is the main constituent of these plaques.
Collins Dictionary of Medicine © Robert M. Youngson 2004, 2005