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Adriano Aguzzi, Mathias Heikenwalder, and Gino Miele

Progress and Problems in the Biology, Diagnostics, and Therapeutics of Prion Diseases. (... the term “prion” was introduced by Stanley Prusiner in 1982 to describe the atypical infectious agent that causes transmissible spongiform encephalopathies, a group of infectious neurodegenerative [brain-destroying] diseases that include scrapie in sheep, Creutzfeldt-Jakob disease in humans, chronic wasting disease in cervids [grazing deerlike animals--deer, elk, etc.], and bovine spongiform encephalopathy in cattle. In this article, the authors review recent progress in the areas of pathogenesis of, diagnostics of, and therapy for prion diseases and [highlight] some conspicuous problems that remain to be addressed in each of these fields.
The authors conclude by saying that there are many aspects of prion biology that remain unclear; we still do not know the precise physical nature of the infectious agent, the molecular and biochemical mechanisms underlying associated neurodegeneration, or the physiological function of the prion protein. The diagnostic tools currently available for prion diseases are significantly less sensitive and satisfactory than those available for other infectious diseases. Additionally, there is a [lack] of therapeutic intervention strategies available for these diseases. However, that said, the last decade or so of prion research has witnessed astounding advances in our knowledge and understanding of basic prion biology, and the field has attracted increasing numbers of researchers from diverse disciplines. Undoubtedly, this trend will trigger further important advances in prion science. Other keywords and phrases -- BSE, CJD, mad cow, sCJD, TSE, vCJD -- from the text of the abstract and the article)

Journal of Clinical Investigation Volume 114, Number 2 (July 15, 2004): pages 153–160.

**The complete text of the article is available at http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pubmed&pubmedid=15254579 (the Web site of the Journal of Clinical Investigation on PubMed Central)**

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Added 4:50 p.m. CT July 23, 2004
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