No Longer Beefeaters /
by Michael W. Paparella




 Shepherd's pie, beef stew or Yorkshire pudding, are being taken off the menu of the British dinner table. Fear of the "mad cow disease" known by the medical term "bovine spongiform encephalopathy", or (BSE) is the reason.
 The disease is thought to be caused by rogue proteins called prions, which eat away at cow's brains, eventually killing them. This nation of beefeaters is worried that the madcow disease may spread to humans.
 This disease (BSE) first broke out in Britian in the early 1980's. Today, infections are appearing in 300 cows per week. Infected cattle are supposed to be destroyed before they reach the market. But since the incubation period for the disease extends 8 to 10 years, some infected cattle show up in slaughterhouses. Public concern in recent months is that contaminated meat is being sold, and eaten.
 This worry over the contaminated meat started with the deaths last year of two teenagers from a malady known as "Creutzfeldt-Jakob Disease", (CJD), the human equivalent of BSE, which is also thought to be caused by prions.
 Human CJD is an extremely rare, incurable disease, only one person in a million gets it. Similar to the bovine form, it has an incubation period of up to 30 years, usually appearing in people 45 years old and older. It is hardly ever seen in those under 30 years of age.
 Moreover, within the last few years four British farmers, all of whom had contact with BSE-infected cows, died of CJD. This might have been dismissed as coincidence had not the incidence of CJD in Britian increased with 55 new cases reported recently.
 Members of the British Parliment debating this issue state as a consensus that there is currently no scientific evidence that BSE can be transmitted to humans, or that eating infected beef causes the disease.
 Medical authorities, on the other hand, refute this statement, saying that they would not eat beef "under any circumstances".
 So far, there have been no reported cases of the disease in American beef stock.
 The causative agent of BSE cannot be destroyed by heat or by radiation. It can pass through filters that would ordinarily catch a virus. No actual virus has ever been found in infected cow's brains. What has been found is a characteristic protein, a "prion", believed to be the true cause of the disease. Prions damage the brain by converting its normal protein counterpart, found in all body cells and known as PrP (prion precursor), into new prions.
 Can cow prions infect humans ? Investigators in British medical schools are trying to find the answer. They are studying genetically engineered mice to carry only the human gene for the PrP. It will take a few years before they know the answer.
 But, for now, do not worry about eating American beef.
 (Portions of this article were gleaned from material in Medicine Watch, May 1996 Issue of Discover Magazine.)


May 5, 1996 Michael W. Paparella The Shore Journal

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