Review: Exchange Magazine

Toronto — Jay Ingram, science popularist, TV host, producer and author has released a book on prions that unveils stunning revelations about disease, the brain and infection. The book, called “FATAL FLAWS: How a Misfolded Protein Baffled Scientists and Changed the Way We Look at the Brain,” published by Harper Collins Canada, argues that, while most people are barely aware of prion diseases—except for perhaps “mad cow” disease— prions are the makings of a revolutionary science that may lead to cures for some of humankind’s most devastating diseases.

 

FATAL FLAWS delves into this scientific mystery, starting with the discovery of Kuru, a disease unique to New Guinea in the 1950s that carried with it whispers of cannibalism. Kuru began a scientific saga until the 1980’s when Nobel Laureate Dr. Stanley Prusiner coined the term “prion”—a misfolded protein—whose existence some of the world’s top scientists still find difficult to accept. This is because the prion (proteinaceous infectious particle) was hypothesized to have a very unique mechanism of infectivity. Unlike other disorders like cancer that use genetic material such as DNA or RNA to cause disease, prions act as a template that “switches” normal cells into toxic disease agents without the use of genetic material. A greater understanding of how the prion converts and causes disease will open the door to developing treatments and preventive options for prion diseases as well as other neurodegenerative disorders, emphasizes Ingram.

 

Today, prions remain controversial with their wide ranging food, health safety and socioeconomic implications. Yet, Ingram argues that these 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 and more recently, chronic trauma encephalopathy cause by repeated concussions.

 

Ingram worked with numerous prion scientists from around the world to produce the book, including many researchers from across the country with PrioNet Canada, the country’s prion disease research network. PrioNet Canada, based in Vancouver, has achieved international attention for scientific discoveries and risk management strategies directed at controlling prion diseases. The organization is directing its research efforts into the development of therapeutic solutions for prion and prion-like diseases of aging, such as Alzheimer’s, Parkinson’s and ALS.