Book Review

Thiago L. Carvalho

Stanley Prusiner:
Madness and Memory: The Discovery of Prions – A New Biological Principle of Disease.

Hardcover: 320 pages
Publisher: Yale Univ Pr (3. Juni 2014)
Language: English
ISBN-10: 0300191146
ISBN-13: 978-0300191141
Price: 23.00 EUR (hardcover), 17.00 EUR (ebook)

I Told You So!

Stanley Prusiner recounts his discovery of prions – and his struggle to convince his peers that they were real.

The historian Robert Conquest endured much slander for his early denunciations of mass famine and murderous purges in Stalin’s USSR. Maligned by academic colleagues as a CIA stooge, Conquest was ultimately vindicated. An apocryphal exchange between him and his publisher had the scholar suggesting as a title for a re-edition of his work I told you so, you ****ing fools.

A similar sentiment pervades every page of Nobel laureate Stanley Prusiner’s memoir, Madness and Memory. “I could have written a bland account of the events leading to the discovery of prions,” Prusiner writes, and then veers off in the opposite direction. This is a good old-fashioned settling of scores and, what’s the point of denying it, it makes for riveting reading. One by one, Prusiner names and nails his critics – very few are lucky enough to remain anonymous like the high school chemistry teacher, who kicks off the book by telling our hero that he “would never be able to comprehend the science”.

Full of wrath: Stanley Prusiner has still to get square with his contemporaries. Painting: Madalena Parreira

Of course, Madness and Memory depicts the defining moment in Prusiner’s life: as a neurology resident, he cares for and watches the rapid decline and death of a sixty-year old Marin County woman diagnosed with Creutzfeldt-Jakob Disease (CJD). The rare, devastating illness had no known cause and no effective treatment. The young Prusiner, who had been actively searching for an important scientific problem to make his own, latches on to CJD and never looks back. Prusiner (and others) soon recognise that CJD can be grouped with other maladies, in particular scrapies in sheep and the puzzling condition known as Kuru that afflicted the Fore people of New Guinea. He joins a small community of researchers attempting to identify infectious agents responsible for these neurodegenerative conditions. The consensus at the time was that they would be slow-acting viruses.

Misfolded protein variants

“Slow” turns out to be a key part of the story, as Prusiner struggles to develop model systems that reduce the scale of the problem from thousands of rodents and many years to something more manageable. We are barely a third of the way through the book when he hits upon his heretical solution: the causative infectious agents are misfolded protein variants that induce misfolding of their native counterparts. The variant, infectious proteins were named prions and they implied a modality of biological inheritance, independent of nucleic acids.

Shifting scientific consensus

Madness and Memory then arrives at its central theme: the long and difficult process of shifting scientific consensus. Prusiner faces a task directly symmetrical to that of Oswald Avery and his team when they demonstrated that genes were made of DNA, and not of contaminating proteins. The last surviving member of Avery’s group, Maclyn MacCarty, makes a brief appearance as an early supporter of prions. Prusiner is ridiculed and ostracised, and the book provides a long list of all those responsible. We see all the stages of resistance to a new idea: denied funding; difficult editors and referees; a paucity of conference invitations; and so on. The main element missing from this very readable story is humour and one is left with the feeling that a bit of it might also have helped Stanley Prusiner in his life. Even in his moments of triumph, Prusiner focuses on the slights he endured. When he wins the 1994 Lasker Award, a crowning achievement in any biomedical career, Prusiner lingers on the negative press coverage.

One reader likened Madness and Memory to “being at a party where someone grabs you by the arm and badgers you for hours”. But in this case, the pushy guy isn’t telling you how to coach Manchester United or solve the Euro crisis in one easy stroke. He is telling you the story of a new biological basis for infectious disease, and he is right. Like Conquest, Prusiner has something important, fundamental to say, and so, he must say it forcefully. The Nobel laureate suffers the slings and arrows of outrageous fortune, and perseveres. Those that persevere with him will be rewarded with a glimpse of revolutionary science in the making.

Letzte Änderungen: 21.11.2014

Information 4

Information 5

Information 6

Information 7

Information 8