title: Working with transmissible spongiform encephalopathy agents.
abstract: The family of illnesses called transmissible spongiform encephalopathies (TSEs), or "prion" diseases, is composed of a small number of human and animal neurodegenerative diseases caused by unique pathogenic agents that are still not fully defined. They are best considered as "protein-misfolding diseases" (together with Alzheimer's disease, Parkinson's disease, and a few other rare examples) resulting from the conversion of a normal body protein into a misfolded amyloid multimer. The pathogenic agents display a unique resistance to conventional disinfection methods and an extraordinary environmental durability, which has led the US Department of Agriculture to designate the causative agent of bovine spongiform encephalopathy as a bioterrorism security threat. In this review, precautions and regulations concerning the handling of TSE agents are discussed in relation to personnel and environmental biosafety.
authors.affiliation: National Institutes of Health, Bethesda, Maryland, USA.
authors.forename: Paul
authors.initials: P
authors.lastname: Brown
authors.forename: Christian R
authors.initials: CR
authors.lastname: Abee
mesh_terms: D000818:Animals; D003264:Containment of Biohazards / Q000379:methods*; D015386:Hazardous Substances; D006801:Humans; D017096:Prion Diseases / Q000209:etiology* / Q000517:prevention & control / Q000635:transmission; D011328:Prions; D012926:Social Control, Formal
chemical_list: D015386:Hazardous Substances; D011328:Prions