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Madness and Memory: The Discovery of Prions - a New Biological Principle of Disease [Hardcover]

Stanley B. Prusiner

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Book Description

3 Jun 2014
In 1997, Stanley B. Prusiner received a Nobel Prize, the world's most prestigious award for achievement in physiology or medicine. That he was the sole recipient of the award for the year was entirely appropriate, for his struggle to identify the agent responsible for ravaging the brains of animals suffering from scrapie and mad cow disease, and of humans with Creutzfeldt-Jakob disease, had been waged largely alone and in some cases in the face of strenuous disagreement. In this book, Prusiner tells the remarkable story of his discovery of prions - infectious proteins that replicate and cause disease but surprisingly contain no genetic material - and reveals how superb and meticulous science is actually practised using talented teams of researchers who persevere. He recounts the frustrations and rewards of years of research and offers fascinating portraits of his peers as they raced to discover the causes of fatal brain diseases. Prusiner's hypothesis, once considered heresy, now stands as accepted science and the basis for developing diagnoses and eventual cures. He closes with a meditation on the legacy of his discovery: What will it take to cure Alzheimer's, Parkinson's, Lou Gehrig's and other devastating diseases of the brain?

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"'Stanley Prusiner is a brilliant scientist whose boldness and tenacity enabled him, against all odds and despite near-universal skepticism, to discover and prove the importance of a new class of disease-producing agents - prions - a discovery as fundamental as that of bacteria and viruses. Prions, by subverting the brain's own proteins, may play a crucial role in Alzheimer's, Parkinson's, and other neurodegenerative diseases - and perhaps afford a clue to their prevention. Madness and Memory is the story of one of the most important discoveries in recent medical history, and it is also a vivid and compelling portrait of a life in science.' (Oliver Sacks)"

About the Author

Stanley Prusiner, M.D., is director of the Institute for Neurodegenerative Diseases and professor of neurology at the University of California, San Francisco. The recipient of an array of scientific honours, he was awarded the Nobel Prize in Physiology/Medicine in 1997. He lives in San Francisco.

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Most Helpful Customer Reviews on (beta) 4.6 out of 5 stars  23 reviews
11 of 11 people found the following review helpful
5.0 out of 5 stars A good read on a revolution in biology 30 Jun 2014
By Simon Manley - Published on
Format:Kindle Edition
The prion story changed our understanding of neurological disease, not only the infectious type {CJD, "mad cow"}, but also the common Alzheimer's dementia. The key to this revolution was realization of the importance of the folding of amino-acid chains for the correct function of proteins and how this could change in disease.

Looking back, it seems curious how resistant the scientific community was to this idea. The fact that proteins had four levels of structure {the amino-acid sequence coded by the gene, the hydrogen bonding, the folding, and the fitting together of separate chains into dimers, trimers, etc} was basic: I learned this in medical school 50 years ago. But the dogma insisted that if something pathologically significant was wrong with a protein, it could only come from the gene, the DNA coding for the amino-acid sequence. And dogma is very had to displace. Anyone advancing a radically new idea will not merely have their data and interpretations challenged: they will be subject to vilification and personal attack.

For the lay reader, this book will give telling insight into the pettiness, and downright viciousness, of scientists competing in a high-profile area. Those of us who are practising scientists ourselves will by-and-large be aware of the sabotage and dirty dealing which is commonplace in scientific politics, so the book will not shock us. Instead, it will give an inspiring insight into how a person with dogged persistence can eventually succeed by sticking to his convictions and answering every criticism with solid data from rigorous experiments.

The writing of the book has attracted comment from some reviewers. It is not up to the standard of literary fiction, but fully adequate to the task of conveying a tale about science. The level of detail is just right. The lay reader with only a smattering of biology will be able to follow, while those of us who are working biologists will be able to fill in the details for ourselves. All in all, an enthralling read on an important topic.
3 of 3 people found the following review helpful
5.0 out of 5 stars Dr Prusiner's "Madness and Memory" an amazing book that explains prions. 23 Jun 2014
By Amy Wong - Published on
Format:Hardcover|Verified Purchase
I chose this rating of five stars since it met my high expectations. I chose to order the book because I heard the author speak about his book, the trials and tribulations and his view that many chronic neurological illnesses such as Parkinson's, which my husband had, could be linked to his field, that of prions. I liked that he spoke about his difficulties and successes which only came after decades and how scientists depend on their aides, money supply and his determination to see things through. I would recommend this book to anyone interested in the brain and its disorders, our unknown part of our body, Alzheimer, CJD, frontal temporal lobe and dementia.
2 of 2 people found the following review helpful
4.0 out of 5 stars Not exactly what I expected 24 Jun 2014
By Molly - Published on
Format:Kindle Edition|Verified Purchase
Prusiner, a great scientist, is rightly honored for the first and very difficult purification of prions (he coined the term) and with insisting and proving, against the tide, that they were infectious proteins with no discernible amount of genetic material, an idea that was prescient for its time. in this book he tells the story of the excruciating efforts it took to obtain enough material for study, including funding struggles to house the huge number of animals needed for the assays. The prion skeptics challenged his ideas for years (some have never given up), leading him to refine his experimental proofs. It's definitely worthwhile reading for this inside look at battles within science. For my taste, too much of the book was taken up with personalities and grudges. I would have preferred more on the science and mechanisms of prion replication (particularly the discovery and experimental manipulation of prions in yeast) and more of a look at the future of prion research. OK for the general reader, though the writing is a bit pedestrian and the experimental detail and departmental politics may deter some.
1 of 1 people found the following review helpful
4.0 out of 5 stars It's not all about the science 8 July 2014
By W. James Dittmar - Published on
How exciting! I love books that take you on a quest for knowledge and answers. This book reminded me of Watson and Crick's classic discussion of their discovery of DNA, "The Double Helix".

What I appreciate about this type of book (and I saw that at least one other reviewer did not explicitly appreciate this) was the mention of the non-scientific parts of scientific endeavors. For example, the reader begins to appreciate that there are many factors other than the skill of the scientist or the thoughtfulness of the experimental approach in determining recognition and acceptance for one's work.

Dr. Prusiner persevered in studying the "prion" in the face of constant criticism from some of his closest colleagues. While I think that science should foster an environment of skepticism, there were times during which it seemed as though personal dislike of Dr. Prusiner interfered with objective scientific analysis and therefore progress.

This book brings to mind the fact that discovering and answering questions about a truly great mystery requires, well, a truly great mystery. And it is not often clear at the outset which mysteries are going to be truly great and which are not. It was indeed fortunate that the prion turned out to be so fascinating and prevalent in neurologic disease. It could have been the case, as you will find out if you read this book, that the infectious agent responsible for spongiform encephalopathy was merely a slow growing virus (which would not have been nearly as interesting)!

I gave this book 4 stars, rather than 5, for two reasons. Firstly, I think the book could have done a better job of explaining some of the scientific or medical concepts. I have some background in biology and medicine so I was able to understand what he meant to say, but I could imagine that someone with a more general background may have difficulty in comprehending some of the details. I thought he didn't explain the purpose or the results of some of his experiments very well, and I had to go back to many of the original papers that he cited to clarify what he meant -- I think it may be difficult to succinctly summarize complicated experiments for a broad audience so I don't see this particular point as being a big issue. Secondly, I kept questioning whether it was truly the science that made people such strong opponents of Dr. Prusiner or whether it was his personality. He, at one point in the book, states that he made a policy not to talk to journalists for a period of 10 years. I think this is a little bit extreme and I wonder whether it is truly the case that all journalists are impatient fools looking to make a news article about a controversial discovery and ignore the science. Still, I can understand his unease with journalists because I did go back and look at some of the articles that he cited and they WERE pretty scathing and uninformed.

This book is certainly worth reading if you would like to read about a quest to solve a fascinating mystery using the tools of modern science and the human brain.
1 of 1 people found the following review helpful
By Waldemar Kalinowski - Published on
Format:Kindle Edition|Verified Purchase
Very few books give the non-scientist a true insight into the world of science as it is practiced in the modern world. Stanley B. Pruisner’s book is written with wit and driven by his unrelenting desire to present the reader with an intelligent description of his decades long quest to unravel the mystery of Kuru, Mad Cow, Creutzweld-Jacob, and host of other terrifying brain diseases.

The crowning moment in this brilliant scientist's career is his discovery of PRIONS, infectious agents which unlike viruses, bacteria, fungi or parasites do not contain nucleic acid (as all living things do) but are complex chemicals (proteins) capable of replicating and causing devastating damage to brain tissue. For his work, Dr. Pruisner received the 1997 Nobel Prize in Medicine.

Although his book delves into the chemistry and the science of the Prion discoveries, it is a wonderful book for any intelligent reader and a must for anyone interested in medicine and health. The author's passionate final chapter should be read first and then again at the end of the book as a call to arms in the war against brain disease.

His conviction that Prion formation in human brains is responsible for a host of other conditions like Parkinson's or Alzheimer's and head trauma induced brain dysfunctions share one underlying common denominator - an ordinary protein which due to an as yet undiscovered process begins to change its physical shape and becomes a Prion, is electrifying.

Hopefully, Dr. Pruisner's instincts and scientific prowess will receive proper government and industry support and his and others’ work with prions will result in cures for these terrifying diseases.
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