Cover image for The pathological protein : mad cow, chronic wasting, and other deadly prion diseases
The pathological protein : mad cow, chronic wasting, and other deadly prion diseases
Yam, Philip.
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Publication Information:
New York : Copernicus, [2003]

Physical Description:
xviii, 284 pages : illustrations ; 24 cm
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RA644.P93 Y35 2003 Adult Non-Fiction Central Closed Stacks

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Prions are an entirely new class of pathogens, and scientists are just beginning to understand them. Although they have plagued humans and animals for 3 centuries, only in the last 2 decades have researchers linked them to diseases like Mad Cow. This book tells the strange story of their discovery, and the medical controversies that swirl around them. The author, Philip Yam, is a well-respected and connected journalist who is now an editor at Scientific American.

Reviews 2

Library Journal Review

When mad cow disease, or bovine spongiform encephalopathy (BSE), and the linked human disease variant Creutzfeldt-Jakob disease (vCJD) made headlines in the early 1990s, many dire predictions and books were issued. Luckily, the epidemic has not surfaced (so far there have been fewer than 100 cases since 1994), but it is still important to learn from past mistakes and understand BSE and other prion (proteinaceous infectious particle) diseases. Because of the ten- to 15-year incubation period, scientists and public health officials are still concerned about the future possibilities of other infected victims. Yam, a writer and editor for Scientific American since 1989, presents a detailed yet readable history of the mad cow scare and other prion diseases that affect humans and animals, such as scrapie. In this regard, his book is similar to Maxime Schwartz's How the Cows Turned Mad in which Schwartz, a French scientist, covers scrapie BSE from a European perspective. Yam gives more coverage to the U.S. reactions to BSE and delves into the problems of chronic wasting disease (CWD) in elk and deer in North America and the research on other prion diseases like fatal familial insomnia (FFI). Recommended for most science collections.-Margaret Henderson, Cold Spring Harbor Lab Lib., NY Technology (c) Copyright 2010. Library Journals LLC, a wholly owned subsidiary of Media Source, Inc. No redistribution permitted.

Choice Review

A mysterious, smaller-than-imaginable, devastating agent leads to sponge-like holes in the brain, and includes heart-wrenching, rapid, certain death. Yam's story recounts in captivating detail the long, arduous investigation of this insidious agent, beginning with famed neurologists Creutzfeldt and Jakob and studies of the kuru epidemics in New Guinea (attributed to the practice of cannibalism). It proceeds to the nationwide slaughter of cattle to prevent the spread of mad cow disease in the UK, then examines the frightening increase in chronic wasting disease (CWD) among American deer. A figure of the human prion protein is included, with locations of common point mutations, as well as a complete description of cellular protein synthesis. A primer in molecular biology is presented in straightforward language and logical sequence; disease-producing, altered prions have long incubation periods, are difficult to detect, are resistant to standard sterilization techniques, and can be transmitted across species. A chapter is devoted to the meat-processing industry and methods that increase disease risk. The potential threat to public health is made clear, but without an alarmist tone. An unforgettable overview, compelling readers to learn more about this unfolding, scientific saga. ^BSumming Up: Highly recommended. Upper-division undergraduates through professionals; two-year technical program students. J. M. Lacey West Chester University of Pennsylvania

Table of Contents

Acknowledgmentsp. xi
Introductionp. xii
Chapter 1 A Death in Devizes: An unusual death in the U.K. marks the arrival of a harrowing new brain diseasep. 1
A Boundless Futurep. 2
Troubling Signsp. 4
"You Don't Die of Depression"p. 8
No Answersp. 9
Chapter 2 One in a Million: A rare disease only gradually becomes recognized as the most common human spongiform encephalopathyp. 13
The Unlucky Fewp. 16
Diagnosing CJDp. 17
Stephen's Case: CJD?p. 20
Chapter 3 The Cannibals' Laughing Death: On a South Pacific island, two pioneering researchers begin to unlock the mysterious epidemic of kurup. 23
Epidemic in the Bushp. 26
A Real-Life M*A*S*H Doctorp. 28
A Lifelong Pursuit Beginsp. 30
Brain Cluesp. 34
Chapter 4 Connecting the Holes: Linking kuru to a disease of sheep enables researchers to experiment with a brain-destroying agentp. 37
An Uncanny Resemblancep. 38
Studying Scrapiep. 41
Trying Transmissionsp. 43
Georgette's Sacrificep. 45
The Kuru-CJD Linkp. 47
An End to an Epidemicp. 48
Nobel Worthyp. 49
Chapter 5 The Birth of the Prion: The unusual mode of attack and biochemical durability of the TSE agent leads to an heretical ideap. 51
A Tough Invaderp. 56
The Elusive Agentp. 58
TSEs' New Playerp. 59
Prion Proposalp. 62
Fatal Filamentsp. 64
The Normal and the Diabolicalp. 66
Chapter 6 Family Curses: Two rare hereditary diseases add support to the prion hypothesis--and challenge it, toop. 69
Coding for Diseasep. 71
The Family That Couldn't Sleepp. 74
One Codon, Two Diseasesp. 79
The Strains Puzzlep. 81
Explaining Strains with Prionsp. 84
Chapter 7 On the Prion Proving Grounds: Research in yeast and other studies show how prions can possess hereditary information and change their shapesp. 87
Prions of Yeastp. 89
From Helix to Sheetp. 92
Cofactors or Cold Fusion?p. 98
The Copper Connectionp. 102
Double Troublep. 104
Chapter 8 Consuming Fears: Modern agriculture enables prions to adapt to a new host, creating the dread mad cow diseasep. 107
Tracking the Sourcep. 110
Forced Cannibalismp. 112
Tackling an Epidemicp. 117
Mad Maxp. 121
The Watcherp. 124
Approaching the Watershedp. 127
Chapter 9 Mad Cow's Human Toll: Figuring out how many people will succumb to variant Creutzfeldt-Jakob disease isn't easy--especially now that BSE has spread around the worldp. 137
Calculating Mortalityp. 138
Mad Sheep Disease?p. 144
Spreading the Madnessp. 145
Chapter 10 Keeping the Madness Out: Several measures help ensure that animal prion diseases do not contaminate the U.S. food supply--but there are gapsp. 153
Cows in the Crosshairsp. 154
Bovine Barricadesp. 157
Breaks in the Firewallp. 160
American Madness?p. 163
In Case of Emergency ...p. 166
Pigs and Sheepp. 168
Chapter 11 Scourge of the Cervids: Chronic wasting disease of deer and elk, once confined to a patch in the Rockies, spreads across the nationp. 171
Out and Aboutp. 176
Venison and Beyondp. 178
Chapter 12 Misadventures in Medicine: Prion diseases spread to humans through medical mishapsp. 183
Surgical Spreadp. 184
Deadly Eyesp. 188
Hazardous Hormonesp. 188
Patch Full of Prionsp. 191
Blood Safetyp. 193
Dental Dangerp. 197
Beyond Beefp. 199
Mystery Pillsp. 202
Chapter 13 Searching for Cures: New hope that the death sentence of prion diseases might someday be liftedp. 205
New Use for Old Drugsp. 206
Rational Thinkingp. 211
Diagnosing Prion Diseasesp. 215
Chapter 14 Laying Odds: Are prion diseases more prevalent than we thought?p. 223
Revisiting Sporadic CJDp. 223
A Case for Undercountingp. 227
Maverick Mayhemp. 232
Menu Choicesp. 234
Man-Made Madnessp. 235
Notesp. 239
Glossaryp. 257
For Further Informationp. 265
Indexp. 269