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Prions
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Introduction |
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| Introduction
Fate and Transport in the Environment Methods for Monitoring in the Environment Methods to Measure Human Exposures |
In 1982 Stanley B. Prusiner published an article in Science claiming to identify the causative agent of scrapie to be a “proteinaceous infectious particle”. He also proposed a new term ‘prion’ (pronounced pree on), to represent the agent’s proteinaceous and infectious nature (1). In 1997 Stanley B. Prusiner received a Nobel Prize for related prion work. The class of diseases are also called Prion Encephalopathies, Spongiform Encephalopathies and Transmissible Spongiform Encephalopathies (TSEs) because of the post mortem appearance of the brain with large vacuoles in the cortex and cerebellum. TSEs share some common characteristics including a prolonged incubation period from a few months to years and fatal neurological signs and symptoms (2). In most cases the victim dies approximately one year after first displaying physical illness (4). Some symptoms include decline of motor skills in all species, visual impairment in some species, and loss of cognitive abilities in humans. At this time there are no known treatments or cures for these diseases. Prions cause a number of specific diseases in animals and humans listed in Table 1.
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