Mad Cow, Chronic Wasting, Other Deadly Prion Diseases (link)

[diamond14]

this article comes from the Chemists, published in 'Chemical and Engineering News' as a book review...this review provides very useful background in prions, mad-cow disease and why bush* policies are NOT protecting America from this crisis....

why is bush* VACATIONING in texas while this crisis spreads???? this article shows how important it is to get bush* up off his ass to take action (instead of spending OUR tax dollars on WAR, bush* should be funding the Chemists' efforts to detect, control, diagnose, and CURE these horrible diseases...we should DEMAND that of the bush* NOW !!!)

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November 10, 2003
Volume 81, Number 45
CENEAR 81 45 pp. 50-51
ISSN 0009-2347


THE INSIDIOUS MARCH OF PRION DISEASES

THE PATHOLOGICAL PROTEIN: Mad Cow, Chronic Wasting, and Other Deadly Prion Diseases, by Philip Yam, Copernicus Books, 2003, 284 pages, $27.50 (ISBN 0-387-95508-9)

REVIEWED BY STEPHEN F. DEALLER

-snips-

To some degree, we treat our agricultural animals as if they are just there to make food and money. So when bovine spongiform encephalopathy (BSE) first appeared in 1986 in the U.K., many of the worries focused on the country's agricultural industry, while the disease was simply pushed to one side. Much of the literature on the subject tells the story of how we gradually realized that maybe humans were at risk, and that perhaps we should worry a bit more.

Yam, an editor for Scientific American, begins this compelling book with the tale of Stephen Churchill, a 19-year-old boy from Southwest England who in 1996 appeared to contract Creutzfeldt-Jakob disease (CJD), a malady normally seen only in elderly people. The disease begins insidiously, with symptoms such as depression and loss of balance. Over several months, Stephen lost his ability to think, see, and move. He finally slipped into a coma and died. Stephen's youth and his disease's resemblance to BSE (mad-cow disease) set off alarm bells around the world, signaling the fear that a lot more disease could be on the way.

The most astounding thing about Churchill's case was that, despite its being a single incident of a previously unknown disease, we could start anticipating the epidemic that might be on its way. The reason--which Yam picks up and explains well--is that the human form of BSE, dubbed variant Creutzfeldt-Jakob disease (vCJD), is just one of a whole range of transmissible spongiform encephalopathies (TSEs). The libraries of data available on the other conditions allowed us to see that they all shared specific factors, such as dementia and long incubation periods. Yam goes back to those other illnesses and shows how the scientific literature has evolved over the previous 95 years to arrive at our current understanding: These diseases are caused by proteins known as prions.


Yam next introduces chronic wasting disease (CWD), the TSE of deer and elk. He points out that the original case of the disease at the zoo in Fort Collins, Colo., in 1967, was treated as an interesting but unimportant blip. However, "between 1970 and 1981, 90% of the deer that stayed at the zoo more than two years died from the disease or had to be euthanized after the onset of symptoms." was clear that the disease was not transmitted in the food and that it seemed to pass from one animal to another, even between deer that had been in pens next door to each other. The nature of CWD transmission is still unknown, and it continues to be an expanding problem.



prion protein cellular model (normal protein)


prion protein scrapie--the "pathological protein"--(notice the TWIST in the red structure)

http://pubs.acs.org/cen/books/8145/8145books.html