Deciphering Prion Diseases with Transgenic Mice

摘要: Prions cause a group of human and animal neurodegenerative diseases that are now classified together because their etiology pathogenesis involve modification the prion protein (PrP) (1). Prion manifest as infectious, genetic, sporadic disorders (Table 16.1). These can be transmitted among mammals by infectious particle designated “prion” (2). Despite intensive searches over past three decades, no nucleic acid has been found within prions (3–6), yet modified isoform host-encoded PrP PrPSc is essential for infectivity (1, 7–10). In fact, considerable experimental data argue composed exclusively PrPSc. Earlier terms used to describe include transmissible encephalopathies, spongiform slow virus (11–13).