Prion Diseases as Transmissible Zoonotic Diseases

Lee, Jeongmin; Kim, Su Yeon; Hwang, Kyu Jam; Ju, Young Ran; Woo, Hee-Jong

At present, a reliable diagnosis of prion disease is possible only through autopsy since there is no approved method for detecting prion levels, which are too low to be detected by any test, in the peripheral nervous systems of live animals or humans. Thus, tissues of the central nervous system, including the brain and spinal cord, obtained at autopsy are used for prion diagnostic tests using immunology-based techniques such as enzyme-linked immunosorbent assay (ELISA), immunohistochemistry, and immunoblotting; a histopathological test is then performed for confirmation. However, the final confirmation is obtained by performing a bioassay to assess the infectivity of the pathogen; this is the most sensitive test and uses a transgenic mouse with the human prion gene to detect pathogenic PrP d by observing the infection. Moreover, DNA sequencing for determination of genetic variations is also considered.