How the Cows Turned Mad Hardcover – Mar 15 2003
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*Starred Review* Two and a half centuries ago, sheep in England started trying to scrape their wool off; in France, to shake uncontrollably. The Brits dubbed their phenomenon scrapie; the French called theirs tremblant. Between then and now, similar conditions in cows and humans were discovered and assigned the group name transmissible spongiform encephalopathies (TSEs): diseases that fill the brain with holes as in a sponge and spread from one organism to another. Their cause eluded researchers until quite recently. SOP since Pasteur sought an invasive bacterium or virus with increasingly powerful tools, which TSE agents eluded. Eventually, evidence pointed to a genetic cause involving transformation of a normal into a deviant gene by another deviant gene introduced orally into the affected organism. You had to eat something from a sick organism to become sick, and once that became popular knowledge after the concurrence of human and bovine TSE cases in England in the 1990s, there was a panic. That reaction seems unjustified; according to Schwartz, TSEs will continue to be a very minor cause of human death. Meanwhile, there may be much to learn from TSE research about such symptomatically similar illnesses as Alzheimer's disease. Writing with immense concentration and clarity, French molecular biologist Schwartz makes the long hunt for the unexpected culprit gene utterly engrossing. Ray Olson
Copyright © American Library Association. All rights reserved
"An excellent overview. . . In an easily understandable way [Schwartz] explains scientific findings."--"British Medical Journal"See all Product Description
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I would note that the book takes the story to 2003. Subsequent developments are easily tracked down with Google. I found current introductary articles on protein folding very helpful.
This is a solid introduction, but read somebody like Richard Rhodes, "Deadly Feasts," for much more detail on the modern end. (Rhodes does a bit much ax-grinding on Prusinder, though.)
Assuming that any cow in England which showed signs of bovine spongiform encephalopathy was an indication that the entire herd had been fed contaminated meat and bone meal, (from "forty-six British plants that until 1988 had converted a total of 1.3 million metric tons of meat and bones into animal feed" p. 147), "the total number of cattle affected by the disease from the beginning of the epidemic until the end of 2000 was nearly two hundred thousand in Great Britain," (p. 151). Since the cow form of the disease and the sheep form act differently in mice who are infected, a grand experimental test was performed to see if any sheep have picked up the BSE form:
"In the summer of 2001, rumors began to circulate to the effect that the BSE agent had been found in sheep; the official outcome was to be announced at the end of the year. Europe's health authorities were in a state of red alert. If the results were positive, drastic steps would have to be taken in the sheep-farming sector. Then, just two days before the outcome was made public, there was a dramatic announcement: The researchers had made a mistake. They had mingled samples of sheep brains with samples of cattle brains--and thus there are still no data on the possible transmission of BSE to sheep in natural conditions." (p. 188).
I have noticed that when people try to assign unique numbers to anything, there is always someone who fails to notice that two of those numbers are not the same. I have even worked with a computer that had so few consecutive numbers in a field that it was not able to tell the difference between numbers that had more than the number of digits in the field. There are forty million sheep in Britain, few of which look like cows, even in that night in which all cows are black, but worse than that: the brain samples might look a lot like brain samples from a cow. This experiment was more than double blind if no one kept tract of how samples were mingled.
I love the word epizootic: "Why was an epizootic--an animal epidemic--declared at one particular time, the early 1980s, and only in the United Kingdom?" (p. 189). It must be related to "the death of six white tigers from the Bristol zoo between 1970 and 1977; they died of what was then diagnosed as a transmissible spongiform encephalopathy, but no one knows what became of the corpses. . . . After all, it isn't often that a cow eats tiger in the way that we eat beef." (p. 190). There are so many things no one knows.
How the Cows Turned Mad details the scientific progressions that have led to our current understanding of prion diseases, more specifically "mad cow disease", in a detective novel format. Beginning with the first veterinary accounts of brain infection in sheep the novel explores the various ways in which the infection could have mutated and been passed to bovine stock. Pasteur's work with physical chemistry and the microscope began to focus the investigations of the brain infections from bacteria to newer, smaller microbes called viruses. Professor Charles Besnoit was the first to apply microscopic investigation to the nervous system of animals infected by scrapie, thereby allowing him to distinguish between the causes of death for the animals. This allowed scientists for the first time to begin to track which diseases were actually killing the animals, while also presenting a view of how the disease progressed to fatality. A combination of work by the scientists Hans Creutzfeldt and Alfons Jakob eventually resulted in the detection of transmissible spongiform encephalopathy in human cases where behavioral and motor activity symptoms were observed. The book explains some of the scientific procedures used to eventually confirm that the fatal brain infections found in sheep, bovine and humans were in fact both similar forms of transmissible spongiform encephalopathy. The disease that for so long had been considered scrapie in sheep, goats and bovine and Creutzfeldt-Jakob disease in humans was found to be one in the same. The cause of scrapie/CJD was finally established by a scientist named Stanley Prusiner, he identified a protein called a prion and found that the protein accumulated in the brains of infected humans and animals to form plaques. Prions were found to propagate by transmitting a misfolded protein state that guides further misfolding of healthy proteins. Since prion diseases have exponential growth rates the accumulation in infected tissue causes damage and eventual cell death. The stability of the folded prion structure also provides a resistance to denaturation via chemical and physical agents making disposal of contamination tissue extremely difficult. Several cases of CJD being contracted via infected bovine lead to wide-spread panic among European and American farmers, in conjunction with public mistrust of bovine meat products. Because of strict measures taken by the United States as well as the United Kingdom the BSE epidemic has largely come to and end, but due to the long incubation period of CJD it will be many years before we can confirm an end to the disease.
How the Cows Turned Mad details the history of the diseases known as transmissible spongiform encephalopathy in a clear manner, although some chapters of the book could be more concise. The writing style of the author is clearly understandable and presented in a format that the lay man could comprehend. Although some chapters, such as 5 and 10 seemed to be longer than necessary to relay the information, the book as a whole was clear and organized. There was not ever a point where the wordiness of the author became a problem with understanding the point of the chapter, but there were sections I felt could have been elaborated on more as well. The inconsistencies were more of the exception to the rule though as I found the book as a whole to be very enjoyable to read. One of the more major issues I had with the book was that it could have used more figures and photographs, there were very few and even as I write this I find it difficult to recall any one of them vividly. Pictures would probably help to solidify and visually represent the disease processes as well as reiterating their similarities. By visually allowing the reader to explore the disease I feel that it would help to audience to become more engrossed in the progression of the disease over time. Figures may also help to organize some of the data that is presented in the body of the book in order to understand trends that were explained. The general tone of the book is somber, which is to be expected when dealing with the neurological infections being discussed, but the final statement of the book is one of hope. A hope that the worst of the infection that has destroyed so many lives around the world is over. The story is a continuing one, but How the Cows Turned Mad will allow many a better understanding of infectious neurological infections.
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