Problematic prions and the history of Mad Cow Disease
Well, folks, it has happened again. A dairy cow from California was recently diagnosed with bovine spongiform encephalopathy (BSE), also known as “mad cow disease.” The cow was already at a rendering plant when the diagnosis was made and, apparently, was never headed toward our food supply. The last confirmed BSE infection in US beef was in 2006, and in total, only four cows have ever tested positive in our country’s entire beef industry. Meanwhile, in just a handful of decades, over a hundred people in the UK have gone “mad” and ultimately died from consuming BSE-tainted beef. In addition, over four million head of cattle have been culled in the UK in an effort to eradicate the problem.
The history of spongiform encephalopathy, however, begins long before the relatively recent BSE crisis — and its victims have included everything from human cannibals to farmed mink. Yet, rarely does science news cover spongiform encephalopathy beyond the context of the grilled burger patty. Burgers are indeed delicious (I prefer mine with BBQ sauce and cheddar cheese), but trust me, the history of spongiform encephalopathy as a disease is way more interesting than this one dairy cow might lead you to believe.
Circa 1920, two German doctors, Hans Gerhard Creutzfeldt and Alfons Maria Jakob, each individually identified the symptoms of spongiform encephalopathy in humans. Hence, the pathology was named Creutzfeldt-Jakob disease (CJD) in their honor. The patients that the doctors studied, however, did not develop their diseases as a result of eating tainted beef. Rather, these patients “spontaneously” developed the condition as the result of a rare (and natural!) genetic anomale.
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