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Fatal Flaws: How a Misfolded Protein Baffled Scientists and Changed the Way We Look at the Brain

by Jay Ingram

Bovine spongiform encephalopathy (BSE), popularly known as Mad Cow Disease, is nothing new to science, but over the past two decades the scale of human tragedy and the huge toll on the beef industry due to outbreaks of this neurological ailment have brought it to the forefront of public awareness.

In his 12th book, science journalist Jay Ingram provides a short history of BSE and a mysterious group of associated diseases. Ingram takes us back to the earliest appearances of related ailments, starting with kuru, a brain disorder ultimately correlated with ritual cannibalism in New Guinea.

Much recent science writing has tended to favour novelistic tactics: strong narrative, interesting characters, and even a plot. Unsurprisingly, Ingram – former host of CBC Radio’s Quirks and Quarks, now co-host of Discovery Channel’s Daily Planet – sticks to a style more reminiscent of broadcast journalism. It works.

Ingram’s subject is controversial (there are no neat conclusions in the book, nor clear heroes or villains), and the morass of details does not lend itself to a narrative approach. Although he walks us through the drama of scientific detective work – and introduces us to some faintly creepy key players along the way – the true protagonist of Fatal Flaws is the scientific method itself.

To the extent that there is a human focus to this book, it is Stanley Prusiner, winner of the 1996 Nobel Prize in Physiology and Medicine. Prusiner coined the word “prion” to refer to the infectious agent in kuru, BSE, and its human equivalent, Creutzfeldt–Jakob disease. His explanation for these diseases – they are caused by a protein with no genetic material involved – is still highly contentious. Yet he stands out from the ranks of researchers, says Ingram, because “science isn’t so narrowly objective; Prusiner’s fondness for this word ‘prion’ reflects his appreciation of the power of language to sell scientific ideas.”

Readers may not be aware of how much red tape entangles much scientific research. Scientists can be as prone to politics, and as keen to cling to prior assumptions, as anyone else – perhaps more. The way Ingram presents the ongoing search for answers surrounding BSE and associated neurological conditions makes for an excellent read.