title: Molecular aspects of disease pathogenesis in the transmissible spongiform encephalopathies.
abstract: The transmissible spongiform encephalopathies (TSE), or prion diseases, are a group of rare, fatal, and transmissible neurodegenerative diseases of mammals for which there are no known viral or bacterial etiological agents. The bovine form of these diseases, bovine spongiform encephalopathy (BSE), has crossed over into humans to cause variant Creutzfeldt-Jakob disease. As a result, BSE and the TSE diseases are now considered a significant threat to human health. Understanding the basic mechanisms of TSE pathogenesis is essential for the development of effective TSE diagnostic tests and anti-TSE therapeutic regimens. This review provides an overview of the molecular mechanisms that underlie this enigmatic group of diseases.
authors.affiliation: Laboratory of Persistent Viral Diseases, National Institute of Allergy and Infectious Diseases, Rocky Mountain Laboratories, 903 S. 4th St., Hamilton, MT 59840, USA. spriola@nih.gov
authors.forename: Suzette A
authors.initials: SA
authors.lastname: Priola
authors.forename: Ina
authors.initials: I
authors.lastname: Vorberg
mesh_terms: D000818:Animals; D006801:Humans; D009154:Mutation / Q000235:genetics; D017096:Prion Diseases / Q000235:genetics / Q000378:metabolism* / Q000473:pathology*; D011328:Prions / Q000737:chemistry / Q000235:genetics / Q000378:metabolism*; D011487:Protein Conformation
chemical_list: D011328:Prions