The Pathological Protein: Mad Cow, Chronic Wasting, and Other Deadly Prion Diseases (Hardcover)

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Key Phrases: eradication zone, pathological prion protein, normal prion protein, New Guinea, Paul Brown, Creutzfeldt Jakob (more...)

Editorial Reviews

Review

. . . a detailed yet readable history of the mad cow scare and other prion diseases. -- Library Journal, June 15, 2003

. . . good at explaining the complex science behind TSEs, as well as the prospects for better diagnosis and treatment. -- The Economist, August 14, 2003

. . . thorough, entertaining and honest. -- The New Republic Online, Summer 2003

An unforgettable overview, compelling readers to learn more about this unfolding, scientific saga. -- Choice, March 2004

Product Description

In 1996, British doctors were horrified to discover that mad cow disease (BSE), an affliction that had been plaguing British cattle for ten years, had jumped the species barrier and was appearing in humans as variant Creutzfeldt-Jakob disease (vCJD). Not unlike the mad cows, victims of vCJD suffer from a degenerative neurological disease that peppers the brain with microscopic holes, causing dementia, loss of motor control, and certain death. What alarms researchers and public health officials worldwide is that the incubation period for vCJD may be as long as 10 or even 15 years, and during this period those infected are symptom-free. And because the disease is so far undetectable except by autopsy, there is no way of knowing with certainty how many people have already been infected. In fact, even travelers who spent time in the U.K. from the early 1980s through the mid-1990s are still considered to be at some risk. What's more, although the U.S. has not detected any mad cows within its borders, there are plenty of "mad deer" running free in several states, and the disease afflicting them is a BSE-type neurological disorder. Called chronic wasting disease (CWD), the illness in these deer has yet to be linked with any human deaths. But given BSE's ability to jump species, there are no guarantees.

In The Pathological Protein, Philip Yam describes how, in this atmosphere of uncertainty, scientists have discovered that the agent of disease in vCJD and a host of other devastating neurological disorders is a bizarre, misshapen version of a protein called a prion. Once introduced into the human neurological system, malformed prions recruit the body's own normal prion proteins, giving them the same pathological ability to destroy brain tissue. Unlike the better-known pathogens that afflict humans -- bacteria, viruses, and parasites -- prions have so far proved resistant to drug therapies and even standard sterilization. No amount of cooking infected meat will prove effective against them.

In a medical detective story with an undercurrent of urgency, Yam describes how the mysterious prion was discovered, how it has been linked to a number of exotic and poorly understood illnesses, and how likely it is that scientists will soon find effective tools for controlling its spread, diagnosing its presence, and treating the devastating disorders it causes.

Philip Yam has been writing and editing for Scientific American since 1989 and is currently the magazine's News Editor. He lives in New York City. This is his first book.

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Product Details

• Hardcover: 304 pages
• Publisher: Springer; 1 edition (June 2003)
• Language: English
• ISBN-10: 0387955089
• ISBN-13: 978-0387955087
• Product Dimensions: 9.3 x 6.4 x 1.2 inches
• Shipping Weight: 1.4 pounds (View shipping rates and policies)
• Average Customer Review: 4.8 out of 5 stars  See all reviews (10 customer reviews)
• Amazon.com Sales Rank: #573,537 in Books (See Bestsellers in Books)

Customer Reviews

Most Helpful Customer Reviews

18 of 18 people found the following review helpful:

5.0 out of 5 stars Easily the best book of its kind, March 25, 2004

By	Stuart MacDiarmid (Wellington, New Zealand) - See all my reviews

Philip Yam's book The Pathological Protein is easily the best book of its kind. Written in clear, simple language for the non-specialist audience, The Pathological Protein is a thoroughly comprehensive, concise and, above all, scientifically accurate review of BSE and related diseases. Yam has been writing and editing for Scientific American since 1989 and this, his first book, demonstrates the high standard to which all science writers ought to aspire.

The first chapter of The Pathological Protein describes, from a very human perspective, the effects of variant Creutzfedt-Jakob disease on one victim, 19 year-old Stephen Churchill, and his family. From this tragedy, Yam then goes on to review the history of CJD and the mysterious diease 'kuru', which reached epidemic proportions amongst the Fore people of Papua-New Guinea because of their cannibalistic funerary rites. After discussing the hereditary transmissible spongiform encephalopathies (TSEs) of humans, outlines what is known of the TSEs of animals. Philip Yam's reviews of scrapie, BSE, transmissible mink encephalopathy and chronic wasting disease are up-to-date, interesting, and extremely readable.

There is an interesting episode related in the book. Carlton Gajdusek had been searching, unsuccesfully, for the cause of kuru. William Hadlow, and American scrapie researcher on a secondment to the United Kingdom, visited the Wellcome Medical Museum in London to look at a display on kuru that Gajdusek had prepared. It was Hadlow who first noticed the very close resemblance between kuru and scrapie. The similarities in epidemiologic features, general clinical pattern and the neurohistologic changes led him to the realisation that these diseases were probably mmebers of the same family. As a result of Hadlow's insight transmission experiments were started which, eventually, led to our current understanding of the TSEs

This book covers the hypotheses for the origins of BSE, the evidence for the link between BSE and vCJD, current methods and problems of diagnosis of the TSEs, and the search for cures. Philip Yam clearly is thoroughly versed in the scientific literature of the TSEs, but also interviewed a broad range of scientists, consumers advocates and regulators. So, he knows what he is writing about, and this is made evident by the clarity and accuracy of his explanations. Although there is no 'dumbing down' of a difficult and complex subject, the author has written a book which makes his subject easily accessible to the non-specialist reader. The book is referenced, well indexed, has a useful glossary and also suggests sources for further information, including the more useful web sites and organisations providing suport and help for families of CJD victims. While the book is written for the interested lay person, I would have no hesitation in recommending Philip Yam's The Pathological Protein to veterinarians and colleagues who want an interesting, thorough and current review of these fascinating diseases.

8 of 9 people found the following review helpful:

5.0 out of 5 stars An engaging and important read!, January 13, 2004

By A Customer

Particularly now that mad cow disease has been found in the US, "The Pathological Protein" is an important and engaging read. The book covers not only mad cow disease and its human form, variant Creutzfeldt-Jakob's disease, but it also covers the underlying cause: misfolded proteins called prions. Yam explains different types of prion diseases and discusses the researchers that have been striving to understand the prion and control the devistating neurological illnesses it causes. Yam discusses the safety of beef today and what could be done to stem the rise in mad cow disease. Well-researched, exceptionally well-written, and highly recommended!

14 of 18 people found the following review helpful:

5.0 out of 5 stars This is a book you should read, August 24, 2003

By A Customer

Protecting the quality of U.S. beef is a concern for each of us individually and for the economy. The Pathological Protein is a good read, a great source of valuable information about prion diseases, and an important guide to some very questionable areas of meat producing.