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Fatal Flaws
Most people have never heard of prions. Indeed, most are only barely aware of the diseases caused by them, except, perhaps, for mad cow disease. Yet prions are the stuff of a revolutionary science—a science that might lead to cures for some of humankind’s most devastating diseases.Fatal Flaws is a scientific detective story about this elusive protein, starting with the discovery of kuru, a disease unique to New Guinea in the 1950s that baffled scientists and carried with it whispers of cannibalism. Kuru began a scientific stampede to seek out the agent of this mysterious disease—the prion—a misfolded protein whose existence some of the world’s top scientists still find difficult to accept. Today, the subject of prions remains controversial, yet the proteins might promise new treatments for some of the most intractable brain diseases, ones that affect millions around the planet, including Parkinson’s, ALS and Alzheimer’s.
272 pages, Kindle Edition
First published May 1, 2012
47 people are currently reading
279 people want to read
About the author
Jay Ingram
56 books39 followersJay Ingram CM (born March 20, 1945) is a Canadian author and broadcaster. He was host of the television show Daily Planet (originally titled @discovery.ca), which airs on Discovery Channel Canada, since the channel's inception in 1995. Ingram's last episode of Daily Planet aired on June 5, 2011. Ingram announced his retirement but stated he will make guest appearances on Daily Planet. He was succeeded by Dan Riskin. His book The End of Memory: A Natural History of Aging and Alzheimer’s is forthcoming from St. Martin's Press in 2015.
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Displaying 1 - 26 of 26 reviews
November 30, 2014
It was incredibly frustrating to read a science book by a guy with such a shallow understanding of the science.
All these cool concepts... with no mention of whether the logical follow up questions have been asked. For instance, if you think Alzheimer's is caused by eating meat, the logical question is, what's the rate of Alzheimer's in lifelong vegetarians?
Also, lots of mentions of theories that are either totally absurd or unfairly misrepresented because they can be dismissed by anybody with a basic understanding of the science. For instance, it makes no sense whatsoever to claim that selection forces in favor of heterozygosity in the past would result in a current over representation of heterozygotes in the population. That's just not how genetics works (anybody who wants a fuller explanation is welcome to send me a message).
Also, I did do some googling as I read this book, and wikipedia, at least, does not have the same view of what has been accepted as science.
All these cool concepts... with no mention of whether the logical follow up questions have been asked. For instance, if you think Alzheimer's is caused by eating meat, the logical question is, what's the rate of Alzheimer's in lifelong vegetarians?
Also, lots of mentions of theories that are either totally absurd or unfairly misrepresented because they can be dismissed by anybody with a basic understanding of the science. For instance, it makes no sense whatsoever to claim that selection forces in favor of heterozygosity in the past would result in a current over representation of heterozygotes in the population. That's just not how genetics works (anybody who wants a fuller explanation is welcome to send me a message).
Also, I did do some googling as I read this book, and wikipedia, at least, does not have the same view of what has been accepted as science.
April 23, 2013
There is no doubt that Jay Ingram knows how to make a story dramatic, and he does so with all guns blazing in Fatal Flaws, the story of the discovery of the (probable) causes of prion-based diseases kuru, scrapie, CJD and BSE.
The first half or more of this book reads wonderfully well at a good pace, exploring the detective story behind the suspicions that these diseases were some how transmittable despite not appearing to involve bacteria or virus – in fact any sign of conventional infection. Ingram focuses on two fascinating areas: what prions are and how they could cause such terrible diseases, and the nature of scientific discovery, warts and all. He profitably spends plenty of time on the less salubrious aspects of academic rivalry and the vastly different approaches of some grandstanding scientists and other solid, behind the scenes workers.
From the offset I thought this was a great book. I have a low tolerance for medical matters, but prions and the nature of their means of attack and transmission are so fascinating that this pushed any medical squeamishness out of the way, as did the biographical detail. After all these appear to be proteins with no DNA component that somehow manage to reproduce, running contrary to what could be regarded as a dogmatic aspect of biology. In a horrible way, prions and their ability to interfere with the way other proteins fold are things of wonder.
Only one thing grated – Ingram insists on telling us over and over again what he is doing and how he is going about it. This is mildly irritating. Also the later part of the book, where he looks at the deer equivalent of BSE and goes through a whole set on chapters on brain diseases that are like prion-based diseases with no obvious prion contribution gets a little tedious and lacks all the storytelling and drive of the earlier section.
All in all, despite these minor failings it’s a gripping read on prions, kuru and BSE, and really gives food for thought on the way we go about science. Recommended.
Review first published on www.popularscience.co.uk and reproduced with permission
The first half or more of this book reads wonderfully well at a good pace, exploring the detective story behind the suspicions that these diseases were some how transmittable despite not appearing to involve bacteria or virus – in fact any sign of conventional infection. Ingram focuses on two fascinating areas: what prions are and how they could cause such terrible diseases, and the nature of scientific discovery, warts and all. He profitably spends plenty of time on the less salubrious aspects of academic rivalry and the vastly different approaches of some grandstanding scientists and other solid, behind the scenes workers.
From the offset I thought this was a great book. I have a low tolerance for medical matters, but prions and the nature of their means of attack and transmission are so fascinating that this pushed any medical squeamishness out of the way, as did the biographical detail. After all these appear to be proteins with no DNA component that somehow manage to reproduce, running contrary to what could be regarded as a dogmatic aspect of biology. In a horrible way, prions and their ability to interfere with the way other proteins fold are things of wonder.
Only one thing grated – Ingram insists on telling us over and over again what he is doing and how he is going about it. This is mildly irritating. Also the later part of the book, where he looks at the deer equivalent of BSE and goes through a whole set on chapters on brain diseases that are like prion-based diseases with no obvious prion contribution gets a little tedious and lacks all the storytelling and drive of the earlier section.
All in all, despite these minor failings it’s a gripping read on prions, kuru and BSE, and really gives food for thought on the way we go about science. Recommended.
Review first published on www.popularscience.co.uk and reproduced with permission
September 25, 2012
Imagine a disease that kills you by rearranging the proteins that make up your brain, creating holes in it and turning your grey matter into a spongy, useless mess. It's incredibly tiny and hardy, making it almost impossible to detect, and resistant to most forms of sterilization. It can lay dormant within a host body from anywhere from two to forty years, and the only real way to know that someone's been infected is via autopsy.
This is a prion. And it's been behind several different diseases in the past century, most notably "mad cow" disease, but also associated diseases such as kuru, Cruetzfeld-Jacob disease, scrapie, and others.
In Fatal Flaws, Ingram manages to lay out the history and pathology of prion disorders, using prose that is technically sound but still easily accessible to a layperson, and manages to present the horrifying reality of prion disorders without seeming sensationalist.
He also paints a refreshingly honest picture about the realities of modern science, and how personality clashes, showmanship, and hubris influence what gets published and what gets attention paid to it. Too often public discussions of science try to show scientists as these demigods of rationality and logic, when really they are just people - smart, dedicated people, but people all the same, with the same feet of clay that the rest of us have.
The history of prion science, as Ingram tells it, is part Sherlock Holmes, part Indiana Jones, and part Michael Crichton. It's a great read for a non-expert with an interest in the field.
This is a prion. And it's been behind several different diseases in the past century, most notably "mad cow" disease, but also associated diseases such as kuru, Cruetzfeld-Jacob disease, scrapie, and others.
In Fatal Flaws, Ingram manages to lay out the history and pathology of prion disorders, using prose that is technically sound but still easily accessible to a layperson, and manages to present the horrifying reality of prion disorders without seeming sensationalist.
He also paints a refreshingly honest picture about the realities of modern science, and how personality clashes, showmanship, and hubris influence what gets published and what gets attention paid to it. Too often public discussions of science try to show scientists as these demigods of rationality and logic, when really they are just people - smart, dedicated people, but people all the same, with the same feet of clay that the rest of us have.
The history of prion science, as Ingram tells it, is part Sherlock Holmes, part Indiana Jones, and part Michael Crichton. It's a great read for a non-expert with an interest in the field.
March 31, 2020
More of a book on the history of prions and the scientists within, Rather than on the biology and physiology of prion diseases.
July 22, 2017
This book starts slowly, somewhat boring, but readily converting into the epic story covering the history of the discovery of the prion proteins: mad cow disease, Kuru disease and similar pathologies, like Parkinson and Alzheimer's disease. Book is not big, but cover rather full story surrounding the discovery - skepticism and controversy, and finely acceptance, but also people and scientific community around the topic. Despite that I am trained pharmchemist I read the book and found tons of new and interesting facts: like standard sterile procedures before mad cow disease do not kill prion protein, or that the incubation period of prion can last up to 20 years, or that the UK government was doing all possible to hide the early facts of mad cow disease! etc... book is fantastic! PS: There are only two chapters about Alzheimer's disease and Parkinson one, but I think you can expand them by linking with previous chapters easily.
September 11, 2012
Some interesting stuff about prion diseases and the science and politics of the research surrounding the theory, but there is still too much uncertainty and too many loose ends for the story to be a particularly satisfying one. Kind of like telling the story of the Copernican revolution and coming to the conclusion that, yes, a lot of the evidence does point to the conclusion that the Earth orbits the Sun, but there are so many inconsistencies in the data that we can't be sure.
July 27, 2024
Twists and turns
I read a lot of books dealing with mostly of biology. This is one of the best in the way it follows a very tangled story with brilliant and strange scientrisrs. It follows the history of the discovery of prion diseases, which are diseases of the brain caused by misfolded proteins that can arise spontaneously or via transmission prion to prion, such as eating meat that has the misfolded proteins. I knew of prion diseases such as Kuru, Kreutzfeld Yakob disease and mad cow disease (BSE), but I had no idea of complexity of the science behind these disease nor of the diversity of prion diseases in animals beside humans, cows, and cervids. (Note that some prominent scientists don’t buy into the prion theory but think that viruses must be causative.) Are Alzheimer’s, Lou Gherig’s, Parkinson’s, and Chronic Traumatic Encephalopatrhy also caused by prions? Read and find out. The book was published in 2012. Still eminently readworthy, bur an upgrade would be great.
I read a lot of books dealing with mostly of biology. This is one of the best in the way it follows a very tangled story with brilliant and strange scientrisrs. It follows the history of the discovery of prion diseases, which are diseases of the brain caused by misfolded proteins that can arise spontaneously or via transmission prion to prion, such as eating meat that has the misfolded proteins. I knew of prion diseases such as Kuru, Kreutzfeld Yakob disease and mad cow disease (BSE), but I had no idea of complexity of the science behind these disease nor of the diversity of prion diseases in animals beside humans, cows, and cervids. (Note that some prominent scientists don’t buy into the prion theory but think that viruses must be causative.) Are Alzheimer’s, Lou Gherig’s, Parkinson’s, and Chronic Traumatic Encephalopatrhy also caused by prions? Read and find out. The book was published in 2012. Still eminently readworthy, bur an upgrade would be great.
April 23, 2024
Fascinating
Have worked in medical field my whole life (RN for 1/2) and have been a hunter. The brain and its' disorders has always fascinated me. Have been following CWD for as long as it's been a thing in the deer population, as well as it's potential to 'jump' species. I process all that I kill not wanting to waste anything. Am much more careful when it comes to the nervous system tissue. It was interesting- but worrisome- to read that the prions can be found in muscle tissue as well . Prion's fascinate me and this book has cleared some misconceptions I have held. On the medical side it certainly seems ironic that many of our other neurological mysteries could be linked especially the dementias' and their apparent causative proteins.
Have worked in medical field my whole life (RN for 1/2) and have been a hunter. The brain and its' disorders has always fascinated me. Have been following CWD for as long as it's been a thing in the deer population, as well as it's potential to 'jump' species. I process all that I kill not wanting to waste anything. Am much more careful when it comes to the nervous system tissue. It was interesting- but worrisome- to read that the prions can be found in muscle tissue as well . Prion's fascinate me and this book has cleared some misconceptions I have held. On the medical side it certainly seems ironic that many of our other neurological mysteries could be linked especially the dementias' and their apparent causative proteins.
July 26, 2024
I had to add a star
This book was way over my head. I’ve had almost no science in my education. And this is pure science. But I read it right through. I have only a small amount of retention of all the words and concepts. But it was easy and enjoyable reading. I’m sorry I’ve reached the end. What more can one say about a science book for those who are not scientists. I repeatedly looked up concepts and names etc to bring things up to date. An excellent book.
This book was way over my head. I’ve had almost no science in my education. And this is pure science. But I read it right through. I have only a small amount of retention of all the words and concepts. But it was easy and enjoyable reading. I’m sorry I’ve reached the end. What more can one say about a science book for those who are not scientists. I repeatedly looked up concepts and names etc to bring things up to date. An excellent book.
April 13, 2024
It was okay at best. There were moments of effective scientific writing. The author wants to seem smarter than he may actually be. Over-dramatized certain things.
I learnt a few things, some of the research is outdated, but for the most part correct, nearly 10 years after publishing
I learnt a few things, some of the research is outdated, but for the most part correct, nearly 10 years after publishing
June 29, 2018
Second half picked up - interesting
July 20, 2024
Well written and extremely interesting. Writer breaks down the topic in to many different parts and describes everything in an informative and engaging manner.
July 1, 2015
"Fatal Flaws" is an enthralling overview of prion diseases and research. I hadn't even heard of prions before reading this book, but despite its morbid nature, found the story of kuru, scrapie, BSE, etc. to be fascinating.
In contrast to many popular science authors, Ingram relies on the merits of the subject itself, as opposed to cheap laughs or funny but ultimately irrelevant anecdotes, to draw in the reader. For him, this is very effective - once I started "Fatal Flaws," I had trouble putting it down. Perhaps his straight-up presentation of the material makes it easy to follow, with few tangential distractions to waylay the reader.
In contrast to many popular science authors, Ingram relies on the merits of the subject itself, as opposed to cheap laughs or funny but ultimately irrelevant anecdotes, to draw in the reader. For him, this is very effective - once I started "Fatal Flaws," I had trouble putting it down. Perhaps his straight-up presentation of the material makes it easy to follow, with few tangential distractions to waylay the reader.
November 4, 2013
I'll admit to not being a biologist, but as a layperson, this seemed like an excellent primer on prions and prion diseases (kuru, scrapie, BSE, CJD and vCJD) as well as a few diseases that may not be related to prions but have related mechanisms (Alzheimers, Parkinsons, Lou Gehrig's). I shall be on the lookout for Mr. Ingram's other books, as this one was well written- clear, concise, interesting, and with well-chosen illustrations.
May 28, 2016
About PRIONS and Mad Cow Disease - BSE.
Easy to read and good “layman’s” explanation of virus’s, proteins and how a cell lives and dies, and written in a fascinating “who done it” way, political cover up's plus hints on Alzheimer’s and other brain disorders.
Author is a renowned writer, with 5 honorary doctorates etc
Easy to read and good “layman’s” explanation of virus’s, proteins and how a cell lives and dies, and written in a fascinating “who done it” way, political cover up's plus hints on Alzheimer’s and other brain disorders.
Author is a renowned writer, with 5 honorary doctorates etc
Read
October 3, 2012Read because I'm doing research on mad cow disease. This is really the story of scientists discovering prion diseases and so there was not as much mad cow info as I would have liked.
I did learn a bit about Creutzfeldt-Jacob and what happens when a person eats a BSE cow though.
Interesting.
I did learn a bit about Creutzfeldt-Jacob and what happens when a person eats a BSE cow though.
Interesting.
September 30, 2013
A good, readable, and compelling account of prion diseases, the discovery of prions, some of the controversy over them, and their potential reach among human diseases. Frankly, a bit terrifying, actually.
July 29, 2014
Baffled! Great book. I didn't realize there was even a controversy that diseases were caused by misfolded proteins. I found it fascinating to learn that diseases can be spread through misfolded proteins. The parts on Cannibalism were creepy.
April 6, 2016
Its a good thing that there are books like this to give a whole different perspective to some of the scientific dogmas that are uncomfortable enough to need guidance as one tries to make sense of it all.
January 12, 2017
An easy read about a fascinating topic -- this book assumes the reader has no real scientific background or knowledge, so very plainly, simply written. Would be a good read for high school or early university biology student, but also good for laypeople.
October 9, 2012
fascinating discussion on prions and their role in CJD, mad cow, and other disorders. As the science isn't settled, the end is a bit disjointed but its still worth the read.
November 16, 2012
A little on the light side for science writing, but I'm finally starting to understand why I'm not allowed to donate blood!
April 19, 2013
Fascinating read about prion diseases and the research into what causes them. Well written and easy to follow.
March 1, 2014
Wished for more detail on protein folding. Pretty light biochemically.. More a history of the epidemiology.
December 28, 2015
I finally understand prions! An excellent, easy read.
January 10, 2016
Well written.
Informative.
Hits the sweet spot of informing without writing down to the reader.
Informative.
Hits the sweet spot of informing without writing down to the reader.
Displaying 1 - 26 of 26 reviews