Cover image for Fatal flaws : how a misfolded protein baffled scientists and changed the way we look at the brain
Title:
Fatal flaws : how a misfolded protein baffled scientists and changed the way we look at the brain
Author:
Ingram, Jay.
Personal Author:
Publication Information:
New Haven : Yale University Press, 2013.
Physical Description:
viii, 282 pages : illustrations ; 22 cm
Summary:
"Discovered and identified as the cause of mad cow disease only three decades ago, the prion is a protein molecule that, when misshapen in the brain, becomes fatal. Novel and controversial, prions have provoked a scientific revolution. They challenge the very foundations of biology: A disease-causing entity with no genetic material at all? A molecule capable of infecting, multiplying, and killing? This book recounts the birth of prion science and the imaginative detective work scientists have undertaken as they struggle to find the answers to devastating brain diseases from mad cow and Creutzfeld-Jakob disease to Parkinson's, Alzheimer's, Lou Gehrig's, and others. As in each of his best-selling books, Jay Ingram here makes complex scientific concepts accessible and shows how little-known events may have profound significance. He describes the development of prion science as a rough-and-tumble affair, with rivals, eccentrics, interfering governments, and brilliantly creative people all playing salient roles. Weaving biology, medicine, human tragedy, discovery, and bitter scientific competition into his account, he reveals the stunning potential of prion science, whose discoveries may unlock the answers to some of humankind's most destructive diseases"--Provided by publisher.
Language:
English
Contents:
The mystery of kuru : a disease like no other -- Barflies and flatworms : how speculation and pure chance advance a new science -- Cannibalism : an answer guaranteed to stir things up -- Igor and Bill : the discoveries that bring kuru to world attention -- The life of a cell : a miraculous, and often precarious, complexity -- The death of a cell : by subterfuge, piracy, or out-and-out assault -- When is a virus not a virus? : when a disease-causing agent reproduces without genes -- Creutzfeldt-Jakob disease : waking up to the potential of a devastating affliction -- Magnificent molecules : the proteins that make life possible -- Protein origami : building the gothic cathedrals of life -- Stanley Prusiner's Heresy : an infectious agent that's a protein and nothing but -- An infectious idea : the campaign for the minds of researchers -- A portrait of the prion : and the experiments that point to their role in the human brain -- Mad cow disease : the uncertain ground where politics and science intersect -- Mad cow in humans : no barrier after all -- The Americas : mad mink, then cows -- Into the wild : deer, elk, moose, and caribou -- Origins : attempting to find where prions come from -- Cats but not dogs : when prions jump the species barrier -- Alzheimer's disease : plaques and tangles but so far no prions -- Parkinson's disease : looking more and more like a prion disease -- Lou Gehrig's disease : the emerging picture of a prion-like process in ALS -- Chronic traumatic encephalopathy : the athletes' plague -- And in the end...
ISBN:
9780300189896
Format :
Book

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Summary

Summary

Discovered and identified as the cause of mad cow disease only three decades ago, the prion is a protein molecule that, when misshapen in the brain, becomes fatal. Novel and controversial, prions have provoked a scientific revolution. They challenge the very foundations of biology: A disease-causing entity with no genetic material at all? A molecule capable of infecting, multiplying, and killing? This book recounts the birth of prion science and the imaginative detective work scientists have undertaken as they struggle to find the answers to devastating brain diseases from mad cow and Creutzfeldt-Jakob disease to Parkinson's, Alzheimer's, Lou Gehrig's, and others. As in each of his best-selling books, Jay Ingram here makes complex scientific concepts accessible and shows how little-known events may have profound significance. He describes the development of prion science as a rough-and-tumble affair, with rivals, eccentrics, interfering governments, and brilliantly creative people all playing salient roles. Weaving biology, medicine, human tragedy, discovery, and bitter scientific competition into his account, he reveals the stunning potential of prion science, whose discoveries may unlock the answers to some of humankind's most destructive diseases.


Author Notes

Jay Ingram is an award-winning science author, writer, and broadcaster. He was co-host and producer of Discovery Channel Canada's Daily Planet from 1995 to 2011, and he is the author of eleven previous books. He lives in Alberta, Canada.


Reviews 2

Publisher's Weekly Review

Science writer Ingram (Theatre of the Mind) provides a thorough overview and update on prion diseases for laypersons. Readers will find the pages turning themselves as they engage with an ongoing medical research enigma. From kuru, the disease that felled New Guineans observing a ghastly carnivorous tradition, to the sheep disease scrapie, known since 1775, to the more recent manifestations of mad cow disease and Creuzfeldt-Jakob Disease in humans, Ingram details the symptoms that mystified researchers for decades until misfolded proteins, named "prions", were identified and hypothesized as the cause of these fatal neurodegenerative diseases. Though their exact roles are still debated, misfolded proteins have now been implicated in Alzheimer's, Parkinson's, ALS, and other human diseases, as well as Chronic Wasting Disease in deer. Ingram balances his narrative with facts and theories to provide a fascinating view into the complexities of cellular life, with several illustrations and photomicrographs to enable the lay reader to visualize what scientists have observed. Noting that puzzles still remain to be solved, Ingram concludes that "prions have revolutionized thinking about how disease can spread... and they still may open doors to the understanding of conditions like Alzheimer's."(Mar.) © Copyright PWxyz, LLC. All rights reserved.


Library Journal Review

Science writer Ingram (The Barmaid's Brain) ranges across far-flung locations and scientific debates as he relates the fascinating details of the title's misfolded proteins, called prions, which have been found to be responsible for numerous degenerative diseases. Ingram begins with the discovery of the disease called kuru in New Guinea in the early 1950s and explores the emerging science around that disease as well as scrapie (spongiform encephalopaths in sheep and goats), mad cow disease (bovine spongiform encephalopathy), Creutzfeld-Jakob disease, and the mysterious misshapen and misfolded proteins (prions) that appear to cause them. He describes how scientists uncovered connections, often owing to nothing but serendipity, among these diseases, thus contributing to the emergence of prion science in spite of rivalries and feuds in recent decades. Just how normal proteins turn into prions is still not entirely clear. The relationship between animal and human prion diseases is complex. The cause of prion diseases in humans can be genetic, sporadic, an infectious agent, or some combination thereof. Identifying outbreaks and their causes has been challenging. Ingram goes on to show how prion science is now being applied to other diseases including Parkinson's and Alzheimer's. Controversy remains about whether prions are the true infectious agents of any of these conditions. Verdict With exceptionally clear scientific explanations and academically sound and readable, often humorous prose, Ingram incorporates the tension and excitement of a gripping thriller while illuminating the human imperfections of science. Highly recommended, this title is essential for neuroscience and infectious disease buffs; almost essential for everyone else, too.-Candice Kail, Columbia Univ. Libs., New York (c) Copyright 2013. Library Journals LLC, a wholly owned subsidiary of Media Source, Inc. No redistribution permitted.


Table of Contents

Introductionp. 1
1 The Mystery of Kuru: A Disease Like No Otherp. 13
2 Barflies and Flatworms: How Speculation and Pure Chance Advance a New Sciencep. 24
3 Cannibalism: An Answer Guaranteed to Stir Things Upp. 32
4 Igor and Bill: The Discoveries That Bring Kuru to World Attentionp. 43
5 The Life of a Cell: A Miraculous, and Often Precarious, Complexityp. 58
6 The Death of a Cell: By Subterfuge, Piracy or Out-and-Out Assaultp. 67
7 When Is a Virus Not a Virus?: When a Disease-Causing Agent Reproduces Without Genesp. 75
8 Creutzfeldt-Jakob Disease: Waking Up to the Potential of a Devastating Afflictionp. 86
9 Magnificent Molecules: The Proteins That Make Life Possiblep. 96
10 Protein Origami: Building the Gothic Cathedrals of Lifep. 103
11 Stanley Prusiner's Heresy: An Infectious Agent That's a Protein and Nothing Butp. 109
12 An Infectious Idea: The Campaign for the Minds of Researchersp. 115
13 A Portrait of the Prion: And the Experiments That Point to Their Role in the Human Brainp. 123
14 Mad Cow Disease: The Uncertain Ground Where Politics and Science Intersectp. 139
15 Mad Cow in Humans: No Barrier After Allp. 151
16 The Americas: Mad Mink, Then Cowsp. 168
17 Into the Wild: Deer, Elk, Moose and Cariboup. 175
18 Origins: Attempting to Find Where Prions Come Fromp. 186
19 Cats but Not Dogs: When Prions Jump the Species Barrierp. 199
20 Alzheimer's Disease: Plaques and Tangles but So Far No Prionsp. 211
21 Parkinson's Disease: Looking More and More Like a Prion Diseasep. 225
22 Lou Gehrig's Disease: The Emerging Picture of a Prion-Like Process in ALSp. 230
23 Chronic Traumatic Encephalopathy: The Athletes' Plaguep. 234
24 And in the End ...p. 239
Acknowledgmentsp. 251
Notesp. 253
Indexp. 271