The Family That Couldn’t Sleep – D. T. Max

An account of prion diseases, among them mad cow, Creutzfeldt-Jakob Disease, and fatal familial insomnia (the family of the title). This book provides lucid explanations of interesting diseases and lays out their frightening implications. It discusses the animal husbandry and butchering practices that spread prion diseases, and it talks about how conflicts of interest within departments of agriculture encourage lackadaisical responses to disease outbreaks. I learned, for instance, that there have been four identified cases of mad cow in the US (the last in April 2006). There is every reason to believe that this is just the beginning.

I was surprised to learn that “prion” refers to one particular protein encoded by a gene on Chromosome 20, rather than a larger class of infectious proteins. The prion protein can fold in two different ways, one healthy and one not, and apparently it can be mis-coded, resulting in heritable forms of prion diseases. Reading the book, I was frustrated that I couldn’t understand how one mis-folded protein could cause different strains of disease. For instance, sporadic CJD, which arises randomly, has a different disease progression than variant CJD, which is caught from cows. Perhaps my confusion is well-founded – the author in his acknowledgements thanks Gary Taubes, “the author of a famous take-down of Stanley Prusiner”, one of the Nobel Prize winners for prion discoveries. Luckily for us, Slate has re-printed the takedown and also printed an update when Prusiner won his Nobel prize. I can’t tell if the science is valid or not, but Taubes (and also Wikipedia) suggests that the prion protein may not cause these diseases after all.

The Family That Couldn't Sleep
D. T. Max
2007
Available from Amazon