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

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

by Philip Yam (Author) "A new resident arrived at Dunstan House Nursing Home in early May, and he was clearly an anomaly ..." (more)
5.0 out of 5 stars  See all reviews (2 customer reviews)
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Product details

  • Hardcover: 304 pages
  • Publisher: Springer (13 May 2003)
  • Language English
  • ISBN-10: 0387955089
  • ISBN-13: 978-0387955087
  • Product Dimensions: 23.6 x 16.3 x 3 cm
  • Average Customer Review: 5.0 out of 5 stars  See all reviews (2 customer reviews)
  • Amazon.co.uk Sales Rank: 337,862 in Books (See Bestsellers in Books)

    Popular in these categories:

    #72 in  Books > Science & Nature > Medicine > Medical Sciences A-Z > Pathology > Microbiology & Virology
    #73 in  Books > Scientific, Technical & Medical > Medicine & Nursing > Medical Sciences A-Z > Pathology > Microbiology & Virology
  • See Complete Table of Contents

Product Description

Product Description

Prions are an entirely new class of pathogens, and scientists are just beginning to understand them. This book tells the strange story of their discovery, and the medical controversies that swirl around them.


From the Back Cover

THE HUMAN VERSION OF MAD COW DISEASE...

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 Creutzfedlt-Jakob disease (vCJD). Not unlike the mad cows, victims of vCJD suffer from a degenerative neurological disorder 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 illneses, 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.


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3 of 3 people found the following review helpful:
5.0 out of 5 stars Easily the best book of its kind, 7 Oct 2003
By S. C. Macdiarmid (Wellington New Zealand) - See all my reviews
(REAL NAME)   
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.

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2 of 4 people found the following review helpful:
5.0 out of 5 stars A view from a relative of a vCJD victim, 18 Sep 2003
By Mr Graham Steel (Scotland, UK) - See all my reviews
I was first alerted to this book about 3 months ago. I have read most of the book now.
The reporting within the book is remarkably accurate and up to date. It pulls in so much information from across the world of the, still, many uncertainties re. TSE issues.
I have sadly learned yesterday of another vCJD victim (alive) in the US. The individual has never been to the UK.
The relavence of this book is of upmost importance if we are to unravel the many unknowns of CJD/BSE and also CWD.

I lost my brother to vCJD in 1999. I am currently Vice-Chair of Human BSE Foundation here in the UK. In essence, we are a support group.
We recently travelled to the US, to "tell or story" at a CJD Conference. We are now working with many other similar Organisations to tackle these outstanding issues. We must campaign globally, if we are so stand any chance in our campaign.

We therefore welcome the importance of The Pathelogical Protien for a number of reasons. The more that people learn accurately about all of this the better for us all.

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