In The Power of Prions, Michel Brahic explores how a mysterious condition transmitted by cannibalism in the 1950s has helped change our understanding of neurodegenerative diseases like Alzheimer’s.
In the 1950s, doctors Carlton Gajdusek and Vincent Zigas trekked over mountains and across rivers to study a mysterious and fatal neurological disease called Kuru. Kuru was killing women and children of the Fore indigenous tribe living in the highlands of New Guinea, and it appeared to spread via cannibalism. It caused tremors, loss of coordination, and neurodegeneration.
After taking brain samples back to the U.S., veterinarian William Hadlow noticed that Kuru resembled Scrapie, a disease in sheep that left small holes in the brain. But unlike other infections caused by viruses, bacteria, or fungi, Scrapie and Kuru appeared to be caused by a chain-reaction of misfolded proteins called prions.
Prions fold in a certain way that causes a chain reaction, making more and more prions that can spread and damage the brain, causing rare neurodegenerative conditions like Creutzfeldt–Jakob disease (the human counterpart to “mad cow” disease — also related to prions). Some researchers think that beta-amyloid and tau proteins — two key hallmarks of Alzheimer’s disease — have prion-like properties too, and that treatments which prevent these proteins from spreading could possibly treat these diseases.
In his new book The Power of Prions, Stanford professor Michel Brahic — a leading researcher on diseases of the central nervous system — tells the story of the mysterious ailment in New Guinea, explains his research into prions, and the surprising discovery that some prions actually help us form memories. What makes the book unique is the explanation of the experiments that helped us understand how prions work and how they spread.
While the prion proteins responsible for diseases are definitively bad, Brahic also explains that these abnormal prions are rare exceptions. Most of the time, prion proteins actually serve vital functions — and they could even have been present at the origin of life itself.
Though the writing is engaging, there is a fair amount of jargon that may trip up readers who don’t have a science background. But, despite it being quite technical at times, the book provides a compelling overview of a relatively new area of scientific study — all packed into less than 200 pages.
