引起人与动物的致死性中枢神经系统疾病 All known prion diseases are fatal Since the immune system does not recognize prions as foreign, no natural protection develops Scrapie in sheep was first described during the 18th century. It has been transmitted to other animals such as mink and cats, and more recently to cows(mad cow disease or bovine 羊搔痒症( scrapie) spongiform encephalopathy, BSE)througl contaminated feedstuff 牛海绵状脑病( spongiform encephalopath In New Guinea. the Fore- people contracted kuru by eating the brains of deceased people Creutzfeldt-Jakob Disease(C]D) frequently rises spontaneously while fatal familial omn lia(FFI) Gerstmann-Straussler Schenker(GSS) disease and 10-15% of c]D are caused by mutations in the gene encoding the prion protein. A new variant CJD, diagnosed in some 20 patients, may have arisen through 人的库鲁病(kuru)、克雅氏病( Creutzfeldt Jakob disease,D)等人的库鲁病(kuru)、克雅氏病(Creutzfeldt Jakob disease, CJD)等 羊搔痒症(scrapie) 牛海绵状脑病(spongiform encephalopathy) 引起人与动物的致死性中枢神经系统疾病