The pathological protein : mad cow, chronic wasting, and other deadly prion diseases / Philip Yam.

Format:

Book

Author/Creator:

Yam, Philip.

Language:

English

Subjects (All):

Prion diseases--Popular works.

Prion diseases.

Genre:

Popular works.

Physical Description:

xviii, 284 pages : illustrations ; 25 cm

Place of Publication:

New York : Copernicus Books, [2003]

Summary:

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 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 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.

Contents:

Chapter 1 A Death in Devizes: An unusual death in the U.K. marks the arrival of a harrowing new brain disease 1

A Boundless Future 2

Troubling Signs 4

"You Don't Die of Depression" 8

No Answers 9

Chapter 2 One in a Million: A rare disease only gradually becomes recognized as the most common human spongiform encephalopathy 13

The Unlucky Few 16

Diagnosing CJD 17

Stephen's Case: CJD? 20

Chapter 3 The Cannibals' Laughing Death: On a South Pacific island, two pioneering researchers begin to unlock the mysterious epidemic of kuru 23

Epidemic in the Bush 26

A Real-Life M*A*S*H Doctor 28

A Lifelong Pursuit Begins 30

Brain Clues 34

Chapter 4 Connecting the Holes: Linking kuru to a disease of sheep enables researchers to experiment with a brain-destroying agent 37

An Uncanny Resemblance 38

Studying Scrapie 41

Trying Transmissions 43

Georgette's Sacrifice 45

The Kuru-CJD Link 47

An End to an Epidemic 48

Nobel Worthy 49

Chapter 5 The Birth of the Prion: The unusual mode of attack and biochemical durability of the TSE agent leads to an heretical idea 51

A Tough Invader 56

The Elusive Agent 58

TSEs' New Player 59

Prion Proposal 62

Fatal Filaments 64

The Normal and the Diabolical 66

Chapter 6 Family Curses: Two rare hereditary diseases add support to the prion hypothesis

and challenge it, too 69

Coding for Disease 71

The Family That Couldn't Sleep 74

One Codon, Two Diseases 79

The Strains Puzzle 81

Explaining Strains with Prions 84

Chapter 7 On the Prion Proving Grounds: Research in yeast and other studies show how prions can possess hereditary information and change their shapes 87

Prions of Yeast 89

From Helix to Sheet 92

Cofactors or Cold Fusion? 98

The Copper Connection 102

Double Trouble 104

Chapter 8 Consuming Fears: Modern agriculture enables prions to adapt to a new host, creating the dread mad cow disease 107

Tracking the Source 110

Forced Cannibalism 112

Tackling an Epidemic 117

Mad Max 121

The Watcher 124

Approaching the Watershed 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 world 137

Calculating Mortality 138

Mad Sheep Disease? 144

Spreading the Madness 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 gaps 153

Cows in the Crosshairs 154

Bovine Barricades 157

Breaks in the Firewall 160

American Madness? 163

In Case of Emergency ... 166

Pigs and Sheep 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 nation 171

Out and About 176

Venison and Beyond 178

Chapter 12 Misadventures in Medicine: Prion diseases spread to humans through medical mishaps 183

Surgical Spread 184

Deadly Eyes 188

Hazardous Hormones 188

Patch Full of Prions 191

Blood Safety 193

Dental Danger 197

Beyond Beef 199

Mystery Pills 202

Chapter 13 Searching for Cures: New hope that the death sentence of prion diseases might someday be lifted 205

New Use for Old Drugs 206

Rational Thinking 211

Diagnosing Prion Diseases 215

Chapter 14 Laying Odds: Are prion diseases more prevalent than we thought? 223

Revisiting Sporadic CJD 223

A Case for Undercounting 227

Maverick Mayhem 232

Menu Choices 234

Man-Made Madness 235.

Notes:

Includes bibliographical references (pages 266-267) and index.

ISBN:

0387955089

OCLC:

51022709