[excerpt] Prions are tiny proteins - smaller even than viruses - and exist in two forms: benign and infectious. The benign form is believed to be involved in the transport of certain minerals throughout the body of mammals. The aberrant form is notable for its weird folding habits.
When the abnormal form comes into contact with normal transporter prions, the normal ones begin a chain-reaction of bizarre folding that ultimately leads to the destruction of neurologic tissue and death.
Mad cow disease belongs to a small group of lethal disorders, caused by an infectious agent that is impervious to heat, ultraviolet light and an array of caustic chemical compounds.
These agents, bizarre proteins known as prions, have been the subject of two major government investigations within the past year. Just last month, the Institute of Medicine, a division of the National Academies, released a report and recommended increased government funding to study these disorders.
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