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The Family that Couldn't Sleep

de D.T. Max

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6782833,705 (3.9)32
For two hundred years a noble Venetian family has suffered from an inherited disease that strikes their members in middle age, stealing their sleep, eating holes in their brains, and ending their lives in a matter of months. In Papua New Guinea, a primitive tribe is nearly obliterated by a sickness whose chief symptom is uncontrollable laughter. Across Europe, millions of sheep rub their fleeces raw before collapsing. In England, cows attack their owners in the milking parlors, while in the American West, thousands of deer starve to death in fields full of grass. What these strange conditions-including fatal familial insomnia, kuru, scrapie, and mad cow disease-share is their cause: prions. Prions are ordinary proteins that sometimes go wrong, resulting in neurological illnesses that are always fatal. Even more mysterious and frightening, prions are almost impossible to destroy because they are not alive and have no DNA-and the diseases they bring are now spreading around the world. In The Family That Couldn't Sleep, essayist and journalist D. T. Max tells the spellbinding story of the prion's hidden past and deadly future. Through exclusive interviews and original archival research, Max explains this story's connection to human greed and ambition-from the Prussian chemist Justus von Liebig, who made cattle meatier by feeding them the flesh of other cows, to New Guinean natives whose custom of eating the brains of the dead nearly wiped them out. The biologists who have investigated these afflictions are just as extraordinary-for example, Daniel Carleton Gajdusek, a self-described "pedagogic pedophiliac pediatrician" who cracked kuru and won the Nobel Prize, and another Nobel winner, Stanley Prusiner, a driven, feared self-promoter who identified the key protein that revolutionized prion study. With remarkable precision, grace, and sympathy, Max-who himself suffers from an inherited neurological illness-explores maladies that have tormented humanity for centuries and gives reason to hope that someday cures will be found. And he eloquently demonstrates that in our relationship to nature and these ailments, we have been our own worst enemy.… (més)
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Es mostren 1-5 de 28 (següent | mostra-les totes)
I learned I had been pronouncing prion like someone from England. This is the first book where the autor included pronunciation.
ANyway I thought he did a good job of integrating the personal stories with the broader scientific research ( )
  cspiwak | Mar 6, 2024 |
A fascinating historical and medical perspective on fatal familial insomnia and prion disorders in general, highlighting historical and modern controversies on these fascinating diseases. Max's strength lies in characterization and the placement of the events occurring in one family with FFI within a historical context. His prose is rich and readable. The subject matter is unspeakably sad, but Max handles a book about rapid neurodegeneration with ease, focusing on the excitement of discovery, the hopes of family and the scientific and medical curiosity evoked by the strange mysteries of prion disorders.

The major flaw is that by attempting to focus on prion disorders in general, what Max covers in breadth often lacks in depth. The discussion of kuru seems to focus on one of the main researcher's pedophilia to a large extent, which seems to occur in place of a real examination of the husband and wife team that did the anthropological work to discover the true origins of the disease. It would be both more salient and more interesting to focus instead on the controversies of cannibalism and how that discovery was made. In addition, Max remarks several times on the similarity between scrapie and FFI to the already discovered hereditary prion disorders CJD and GSS, without ever really discussing the discovery of those conditions. Since one of the stated goals of the book is to bring about public awareness and support for research to inherited prion disorders, more exploration of these two diseases would have added a lot, in addition to enriching the history of the field. ( )
  settingshadow | Aug 19, 2023 |
A friend has been reminding me to distinguish between an interesting topic and a topic I'd actually want to read a full-length book about. When I first heard of FFI I was fascinated, but honestly I'm not sure what 250 pages just on that would look like - probably science I wouldn't quite understand. Luckily this particular book covers more than FFI, giving a history of research into prions and prion disease research, with forays into kuru (always memorable if you've studied anthropology or traveled the south pacific) and scrapie. It was additionally interesting to read about mad cow, because I remember it, but wasn't at an age where I read the news closely, nevermind scientific papers. Overall very readable and interesting, despite the rather accepting treatment of Gajdusek. ( )
  Kiramke | Jun 27, 2023 |
Riveting. Loved it.
1 vota sallypursell | Jun 7, 2019 |
An excellent history of prion diseases, framed by the story of an Italian family with a genetic prion disease, fatal familial insomnia, that kills by exhaustion. I'm equal parts fascinated by this disease's potential to illuminate the way sleep works, and horrified by the very thought of prion diseases. ( )
1 vota jen.e.moore | Aug 9, 2017 |
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Nom de l'autorCàrrecTipus d'autorObra?Estat
D.T. Maxautor primaritotes les edicionscalculat
Blake, MartyAutor de la cobertaautor secundarialgunes edicionsconfirmat
Gardner, GroverNarradorautor secundarialgunes edicionsconfirmat
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Si et cal més ajuda, mira la pàgina d'ajuda del coneixement compartit.
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Now all my hours are trances;

And all my nightly dreams

Are where the dark eye glances,

And where thy footstep gleams,

In what ethereal dances,

By what Italian streams.

—EDGAR ALLAN POE, “The Assignation”
Protein, so far as we know, does not replicate itself all by itself, not on this planet anyway. Looked at this way, the [prion] seems the strangest thing in all biology and, until someone in some laboratory figures out what it is, a candidate for Modern Wonder.

—LEWIS THOMAS
Dedicatòria
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For Sarah

Dove andrai tu andrò anch’io

e dove starai tu io pure starò
Primeres paraules
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In October 1997, Stanley Prusiner, a professor at the University of California–San Francisco who had spent twenty-five years studying prions, went to Stockholm to receive the prize he called “the big one” from the King of Sweden.
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Cap

For two hundred years a noble Venetian family has suffered from an inherited disease that strikes their members in middle age, stealing their sleep, eating holes in their brains, and ending their lives in a matter of months. In Papua New Guinea, a primitive tribe is nearly obliterated by a sickness whose chief symptom is uncontrollable laughter. Across Europe, millions of sheep rub their fleeces raw before collapsing. In England, cows attack their owners in the milking parlors, while in the American West, thousands of deer starve to death in fields full of grass. What these strange conditions-including fatal familial insomnia, kuru, scrapie, and mad cow disease-share is their cause: prions. Prions are ordinary proteins that sometimes go wrong, resulting in neurological illnesses that are always fatal. Even more mysterious and frightening, prions are almost impossible to destroy because they are not alive and have no DNA-and the diseases they bring are now spreading around the world. In The Family That Couldn't Sleep, essayist and journalist D. T. Max tells the spellbinding story of the prion's hidden past and deadly future. Through exclusive interviews and original archival research, Max explains this story's connection to human greed and ambition-from the Prussian chemist Justus von Liebig, who made cattle meatier by feeding them the flesh of other cows, to New Guinean natives whose custom of eating the brains of the dead nearly wiped them out. The biologists who have investigated these afflictions are just as extraordinary-for example, Daniel Carleton Gajdusek, a self-described "pedagogic pedophiliac pediatrician" who cracked kuru and won the Nobel Prize, and another Nobel winner, Stanley Prusiner, a driven, feared self-promoter who identified the key protein that revolutionized prion study. With remarkable precision, grace, and sympathy, Max-who himself suffers from an inherited neurological illness-explores maladies that have tormented humanity for centuries and gives reason to hope that someday cures will be found. And he eloquently demonstrates that in our relationship to nature and these ailments, we have been our own worst enemy.

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