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The pathological protein : mad cow, chronic wasting, and other deadly prion diseases/, Philip Yam
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The Pathological Protein: Mad Cow, Chronic Wasting, and Other Deadly Prion Diseases
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Add a tag Cancel Prion. Immediately, the reader is bombarded by warning signs. The cover is decorated by a background of case studies, all fatal. The title of the book looks like a red hazardous materials sticker taped across a Holstein.
Prion Diseases | CDC
The first page of the introduction tells us Yam is at risk by going to a dentist. The first chapter relates the tragic death of a feisty young Briton with a promising future. Yet The Pathological Protein is a non-alarmist, engagingly-written, current book that can help us live with our choices to eat or eschew meat. The news editor for Scientific American , Yam writes to the level of the scientifically-curious, not the scientifically-trained, and inspires confidence by his grasp of the subject as he brings rather than drags us along.
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He uses direct quotes and diagrams, often giving parenthetical explanations for medical terms, a basis for an epidemiological prediction, or a context for a comment. After going through the initial scare tactics, which we all have experienced to some degree thanks to incomplete media coverage and our own fear of the unknown, Yam shows us which theories experimentation supports, while always recognizing that miniscule risk is not zero risk.
This review focuses on kuru and fatal familial insomnia, with some discussion of Creutzfeldt-Jakob disease. The transmission of these diseases is of special interest because of the noninflammatory nature of the clinical-pathologic syndrome, the long incubation period "slow" virus infection , and the unusual nature of the transmissible agent, the prion.
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The importance of these diseases was demonstrated by the receipt of the Nobel Prize in and by Gajdusek and Prusiner respectively. Fatal familial insomnia was first described in Lugaresi et al In fatal familial insomnia patients were demonstrated to carry a mutation in the PrP gene, PRNP , as is the case with familial Creutzfeldt-Jakob disease; for this reason, fatal familial insomnia was proposed to be a prion disease Medori et al The transmission of fatal familial insomnia to experimental animals has confirmed its inclusion in this group of diseases Tateishi et al A noninherited disease, called fatal sporadic insomnia, with a similar clinical and pathological phenotype to fatal familial insomnia has also been described Mastrianni et al ; Abu-Rumeileh et al In addition, the recognition of chronic wasting disease as a prion disease of deer and elk in the United States has focused attention on prion diseases.
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