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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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The Pathological Protein: Mad Cow, Chronic Wasting, and Other Deadly Prion Diseases Philip Yam COPERNICUS BOOKS yam00FM 3/17/03 12:43 PM Page i The Pathological Protein This page intentionally left blank yam00FM 3/17/03 12:43 PM Page iii The Pathological Protein Mad Cow, Chronic Wasting, and Other Deadly Prion Diseases Philip Yam copernicus books An Imprint of Springer-Verlag yam00FM 3/17/03 12:43 PM Page iv © 2003 Philip Yam All rights reserved. No part of this publication may be reproduced, stored in a retrieval system, or transmitted, in any form or by any means, electronic, mechanical, photocopying, recording, or otherwise, without the prior written permission of the publisher. Published in the United States by Copernicus Books, an imprint of Springer-Verlag New York, Inc. A member of BertelsmannSpringer Science+Business Media GmbH Copernicus Books 37 East 7th Street New York, NY 10003 www.copernicusbooks.com Book design and line art by Jordan Rosenblum. Library of Congress Cataloging-in-Publication Data Yam, Philip. The pathological protein: mad cow, chronic wasting, and other deadly prion diseases / Philip Yam. p. cm. Includes bib