Mad Cows and Cannibals Each year thousands of illegal aliens breach the U.S.

borders and disappear into the heartland. The people responsible for homeland security and immigration seem unable to stop them or find them. But in December 2003, one undesirable immigrant was spotted, detained and interrogated with the kind of ruthlessness usually reserved for members of Al Queda. The suspect alien was a disabled, six-year-old diary cow from Canada. This cow was a “downer;” it was clearly sick, and stumbling, shaking cows are a possible sign of Mad Cow Disease. Canadian cows used to cross the border with the ease of two-legged tourists, but the border was closed to live cattle imports 19 months earlier when an Alberta cow was found to have Mad Cow Disease. Now the presence of a possible second case, imported into Washington State, suggested the disease was present in Canadian cattle and some of those cattle were now in the U.S. That was not good news for public health, international trade or the beef industry. (A third Alberta cow has since been found.) Mad Cow Disease is not a bovine personality disorder. Rather, it is a rare—and fatal— neurological disease. It is an extraordinary disease both for the way it is transmitted and the nature of the agent that causes the disease. Until 1982, all infectious agents were assumed to be living creatures like bacteria and parasites or non-living complexes like viruses. The agent of Mad Cow Disease (or Bovine Spongiform Encephalopathy) turned out to be none of the usual suspects. Instead, it is a protein: a self-replicating, virtually indestructible, infectious protein. Worse, it is a normal host protein found in normal brain tissue. The man who made this heretical discovery, Stanley Prusiner, called these infectious proteins, “prions.”

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How could a normal brain protein cause a devastating, fatal disease? And how could it be transmitted from person to person or cow to cow? No one is sure what prions normally do, but they are clearly important. Like all proteins, prions have distinct shapes; change the shape and all hell breaks loose. Sometimes a random prion may assume an incorrect shape and cause other normal prions to take on that shape. That leads to sponge-like holes in the brain, debilitating illness, dementia, and death. As an analogy, imagine driving your car down a crowded highway. Someone in front of you slams on the brakes and you hit that car. Suddenly your car has taken on a new shape, which may affect its overall function. The cars behind you will be taking on interesting new shapes too. The end result of all these shape-changed cars is a clogged, dysfunctional highway. So what causes that first car to slam on its brakes? What causes that first prion to alter its normal shape? Probably random chance. Things just happen. That’s probably why a very rare human disease called Creutzfledt-Jakob disease (CJD) pops up among less than five people per billion per year. Human CJD looks just like Mad Cow Disease. That suggests man and cow suffer the same disease from the same source: misshaped prions. In the 1990’s Great Britain had an epidemic of Mad Cow Disease. Over a million cattle acquired the disease. Where did they get it? Probably from sheep. Sheep sometimes develop a disease called scrapies, which is another prion disease. Leftover sheep parts were rendered into protein supplements for cattle feed. The indestructible sheep prions survived the trip from sheep to cows where they started causing BSE in cattle. The epidemic among cattle was amplified by rendering leftover beef products back into cattle feed and giving it to more cows. In effect, cows were eating cows and passing around disease-causing prions.

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Over the next few years, 147 beef-eating Brits developed a fatal brain disease. The disease was called “new variant CJD” (vCJD) to distinguish it from the rare, natural CJD. Suddenly, CJD wasn’t so rare and the once obscure prion was now front page news. Cattle parts containing high concentrations of prions—brains, spinal cords, tonsils, eyes and other nerve tissue—had slipped into human food products. This is why officials on both sides of the U.S.-Canada border are nervous. Some cattle shipments are scheduled to resume in March. The USDA asked the Harvard Center for Risk Analysis to determine how vulnerable the U.S. is to BSE. The Center concluded we are “highly resistant to any introduction of BSE.” Of course, that resistance depends on compliance with new feed regulations and closing loopholes allowing cattle to be fed supplements of cow blood, restaurant scraps and chicken litter. Half a century ago, New Guinea tribesmen suffered from a fatal disease called kuru. It was a prion disease. They got it from eating the brains of their dead relatives. When they stopped being cannibals, kuru disappeared. When we stop turning cows into cannibals BSE and vCJD will disappear too.

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