朊蛋白病是由异常朊蛋白在脑内蓄积所致。本文综述了朊蛋白的生化特性、异常朊蛋白形成机制、正常朊蛋白向异常朊蛋白转变机制、朊蛋白株和朊蛋白病在不同种属间传递的特点。 Prion diseases are caused by accumulation of abnormal prion protein in the brain. This review summarizes the biochemical characteristics of prion protein, the mechanism of abnormal prion protein formation, the mechanism of prion protein transformation to abnormal prion protein, and the transfer of prion protein and prion protein disease among different species.