Fatal flaws
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"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.
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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.
