REVIEW: The Pathological Protein
More than just mad cows
A balanced look at the current state of prion research

By Trina Smith


The Pathological Protein: Mad Cow, Chronic Wasting, and Other Deadly Prion Diseases

By: Philip Yam

Copernicus Books, 2003, 284 pp.
ISBN 0-387-95508-9, $27.50

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January 29, 2004: In this book Philip Yam gives a timely and thorough examination of TSEs—transmissible spongiform encephalopathies—the communicable diseases named for their tendency to produce holes in the brain (encephalon), giving it a sponge-like appearance. Given the recent publicity about the Washington Holstein positively diagnosed with B(ovine)SE, commonly called mad cow disease, surely Yam’s work will be appreciated by and informative to a wide cross-section of the concerned public.

Picking up the book is like reliving the first time one heard about TSEs. Immediately, the reader is bombarded by warning signs. The cover is decorated by a background of case studies, all fatal. The title of the book looks like a red hazardous materials sticker taped across a Holstein. The first page of the introduction tells us Yam is at risk by going to a dentist. The first chapter relates the tragic death of a feisty young Briton with a promising future. Yet The Pathological Protein is a non-alarmist, engagingly-written, current book that can help us live with our choices to eat or eschew meat.

The news editor for Scientific American, Yam writes to the level of the scientifically-curious, not the scientifically-trained, and inspires confidence by his grasp of the subject as he brings rather than drags us along. He uses direct quotes and diagrams, often giving parenthetical explanations for medical terms, a basis for an epidemiological prediction, or a context for a comment. After going through the initial scare tactics, which we all have experienced to some degree thanks to incomplete media coverage and our own fear of the unknown, Yam shows us which theories experimentation supports, while always recognizing that miniscule risk is not zero risk. A one-in-a-million chance of contracting a disease still affects 100 percent of the individual who contracts it, and each individual must choose the level of personal risk he or she finds acceptable. Yam states that his hope is to help inform that personal decision.

Through a peripatetic tour of global research on various TSEs (afflicting sheep, humans, goats, elk, deer, mink, hamsters, mice, cows, chimps and others, plus an attempt to inoculate an alligator), Yam introduces us to an assorted cast of researchers and policy-makers. He forces even the most flamboyant characters to take a back seat to their own often amazing work, and makes a few tactful allusions to the politics of science. Nobel-hunting and grant-funding on the basis of what some view as scientifically trendy prove that even science can’t exist in a cultural vacuum. An economic vacuum is even less plausible; trade embargoes, oil prices, various animal product industries and even the WTO influence whether or not science is funded or scientific findings are implemented in policies. Nevertheless, a growing understanding of the many TSEs—strains within a species as well as diseases across species—owes its development to dedicated, interdisciplinary researchers, including anthropologists, veterinarians, physicians, nuclear physicists, biochemists and mathematicians.

Yam gives us the prevalent views along with dissenting opinions, and, where possible, supports them scientifically. Today, the majority of scientists believe that TSEs are caused and transmitted by a malformed prion (rhymes with neon, not lion), a proteinaceous infectious agent, that not only causes holes in brains, but also puts holes into some traditionally-held beliefs. Prions seem to be without nucleic acids (DNA and RNA, the carriers of genetic information), so they defy the generality that only nucleic acids can dictate how a protein is structured. Prion diseases seem to be both infectious and hereditary. Many brilliant people have built careers on the impossibility of that statement, but now mounting evidence shows that it’s not only possible, but the best theory going. Many minds are contributing to explanations for prion synthesis, how and why it goes awry, how non-pathological prions function in the body, and how some baker’s yeast supports prion theory. Some people are even working on strategies to cure TSEs before anyone fully understands them. Yam lists these strategies on p. 215. I have renewed respect for massive theoretical creativity in the face of what looks like a brick wall of data.

As I neared the end of The Pathological Protein, I thought I had the book pegged as a well-researched story of “unfair” amoral nature visiting upon us a new plague, with naught but a clever cadre of scientists to protect us. However, the book surprised me in the last three pages. Early on, he made a few passing references to human responsibility for feeding cows and mink infected animal remains, and for transporting C(hronic)W(asting)D(isease)-carrying elk to hunting grounds for sport, but he gives human-imposed natural imbalance center stage in his last lines. As he ties post-WWII ideas of efficiency to unrealistic expectations about the price of meat, to questions about the value and quality of animals' lives, I found myself hoping, Please, American public, read this book.

Trina Smith has recently read two other books on TSEs, and very highly recommends The Pathological Protein as the best of the three. Please consider, she suggests, buying a copy, reading it, and then donating it to your local public library.