THE PIONEER MAD COW RESEARCHERS

Here is the story of the men and women who unraveled the mystery of prions and mad cow disease. We owe a lot to them. They did most of their work at a time when their efforts were not always appreciated.

Although the earliest researchers could not figure out what caused Kuru, Dr. Carleton Gajdusek, a virologist employed by the National Institutes of Health (NIH), carefully examined the Fore people in the early 1950s. Back in Maryland at the NIH, the similarity between Kuru and CJD, an extremely rare disease, was recognized.

Dr. William Hadlow, a scrapie expert, later found that Kuru brains were like those in scrapie sheep. He wrote a letter to Lancet, the British medical journal, suggesting that a researcher check to see if the condition was transmissible. In order to determine that, Gajdusek started injecting chimps and monkeys with the ground brains of Fore tribeswomen. By 1965, they had shown that Kuru was transmissible. After extending the experiments, Gajdusek demonstrated that scrapie, Kuru and CJD could all spread and kill in the same manner. He received a Nobel Prize for his work. But the pathogens causing the disease were still unknown.

CJD (the human form of mad cow disease) is a horrible sickness that usually involves deterioration into blindness, dementia, and loss of nerve function. Both Kuru and CJD were always fatal, and resulted in corpses whose brains were riddled with holes; hence the name, "spongiform" (sponge shaped).

To this day, few scientists want to go near CJD. They prefer not to do research on it, and pathologists avoid undertaking postmortem examinations of patients suspected of death by CJD. The problem is that none of the common solvents and heat sterilization techniques used in bacterial research will decontaminate the disease-causing agent or, for that matter, anything it touches!

In addition, the infective agent is extremely infectious. In those instances in which they do examine remains, researchers wear masks, goggles, gloves, boots, caps, operating gowns, and plastic aprons; all of these can be destroyed afterward!

But none of this was known by scientists in earlier decades in the U.S. and Britain when they tried to figure out what Kuru was. When they failed, the test samples were dumped down the sink drain or tossed out. Surely, they thought, there is no infective agent present.

But something was there. And now, in its most virulent form, it was outside the U.S. and British laboratories, infecting passing wildlife.

After losing a patient to CJD, Stanley B. Prusiner, a biochemist at the University of California, worked on the problem and gradually developed a theory that the infective agent in all these spongiform encephalopathies was not a virus but a kind of abnormal protein, to which he gave the name, "prion" (Richard Rhodes, Deadly Feasts, pp. 121-123).

His theory was based on the work of two earlier researchers, Tikvah Alper and Carleton Gajdusek, who speculated that the infectious agent in scrapie might be something which lacked nucleic acid (Richard Rhodes, pp. 160-162). That seemed impossible, yet extracts of scrapie-infected brains retained their ability to transmit the disease—even after being radiated to a degree which would destroy nucleic acid (Prusiner, "The Prion Diseases," Scientific American, January 1995, p. 49). (Nucleic acid is the substance containing DNA, and is present in all viruses.)

Prusiner eventually identified 15 of the amino acids at one end of a prion. Checking deeper, he found that "normal proteins" were in a single gene in both man and all animals that he tested (ibid.). The normal ones were no problem. But, due to some strange circumstance, they could become perverted into something terrible—the spongiform diseases.

Eventually, Prusiner figured out that the wrong type of protein is able to make contact with normal proteins—and contaminate them! In this way, the disease spreads throughout the brain, as well as into nerve, blood, and bone cells throughout the body.

A normal protein is folded in a certain manner. When the abnormal protein makes contact with it, the normal protein unfolds—and then refolds in the same damaged pattern that abnormal proteins (prions) are in! That is how the prion propagates itself (Prusiner, p. 52).

Sounds strange? It surely is. An amazing new plague for our time in history. Some say it will eventually be worse than AIDS; for many people eat meat, and the meat they are eating was fattened on diseased, dead animals—just like those natives in New Guinea who ate their dead relatives. People should not eat people, and cows should not eat cows. The result, for all concerned, is mad cow disease.

Prusiner had identified an entirely new type of disease agent. But, next, we go to Richard Marsh, a researcher at the University of Wisconsin.

The British Government maintained that cattle were a "dead-end host" of spongiform disease. By that, they meant that cows were getting spongiform spontaneously, and could not transmit it to anyone who ate them. Marsh did not believe that theory.

Marsh suspected, rightly enough, that the British Government was saying this to keep the public from panic—and keep them buying British beef. The theory also made it convenient to keep feeding animals to the British cattle.

To keep the money rolling in, the cycle of ever-increasing doom for an entire nation was permitted to continue.

Are we doing something similar right here in America?

Marsh (who died in 1997) took material out of the brains of infected mink and placed it into the brains of two Holstein steer calves. A year and a half later, the steers suddenly fell over dead. Then Marsh transferred some of their brains back into mink which then died of TME (transmissible mink encephalopathy, the mink form of the disease).

Based on Marsh’s research, it was obvious that mad cow disease could, indeed, "jump the species barrier"—and infect human beings!

Further research eventually disclosed that the U.S. form of BSE is called "downer cow syndrome." Instead of wobbling before dying (as British cows do), U.S. cows with the disease just fall over dead.

It so happens that slaughterhouses, both in Britain and the U.S., do not accept cattle unless they arrive alive and are able to walk. So cattle which die of BSE—in both the U.S. and Britain—are sold to rendering plants, which cook them at 280o F., and turn them into feed pellets, to be fed to feeder cattle at the feedlots, which are then shipped to the slaughterhouses, prior to being placed on your table to eat.

There you have the whole procedure.

Add to this the fact that mad cow disease can linger in humans for years before it manifests itself. AIDS kills within eight to 15 years. But CJD can go as long as 35 to 42 years, or more, before killing its victim. How do we know this to be true? because some Kuru victims died as much as 42 or more years—after eating a dead relative. (Most lived 10 to 35 years after eating a relative.)

William Gordon, a British researcher, decided to duplicate Marsh’s research. He injected scrapie into many a variety of animals (including goats and mice) and caused their death from the same disease (Rhodes, p. 121).

But certain significant facts should be noted: Gordon discovered (1) that the amount of infected material, fed or injected in the animal, determined how fast it developed the full-blown disease. (2) Gordon could infect animals, not only with blood or brain, but also with muscle tissue.

Do you know what it is that you eat, when you eat meat? You are eating muscle tissue. That is what is sold to you at the meat counter of your corner grocery store or served to you in a wrapper at your nearby fast-food restaurant.

So we have here a mammoth problem: For years, the British and U.S. Governments sided with their meat industries, in declaring that mad cow disease could not "jump the species barrier"; yet Marsh and Gordon had shown that it could. That posed the question as to whether it could also jump the barrier from the animals—and infect our brains.

Another question was whether mad cow disease was in America. The official word has been that it definitely is not.

Yet thousands of "downer cows," with spongiform brains, drop dead in the U.S. every year. (They are immediately ground up and turned into cattle, sheep, hog, and chicken feed.) It is also known that a variety of wild animals in our continent also have a type of the disease. Yet the official statement remains that there is no need to worry; mad cow is only in Europe.

One researcher asked Marsh how the mink used in his experiment contracted mad cow disease. He replied that he could not say for certain, but that those mink had been fed the carcases of downer cows (H.F. Lyman, Mad Cowboy, p. 88).

A major breakthrough occurred in October 1996, when Dr. John Collinge reported in the British journal, Nature, on a new biochemical test for prions. Using it, he found that the biological "fingerprints" of prions in patients were identical to those in laboratory animals with BSE. —Finally, this is definite proof that both humans and animals had mad cow disease! (John Collinge, et al., "Molecular Analysis of Prion Strain Variation and the Etiology [cause] of ‘New Variant’ CJD," Nature October 24, 1996).

A year later, he reported on CJD samples taken from people who had died, which, when placed in test animals, produced BSE! BSE transferred from a mad cow to test animals produced the same BSE. This was the final proof (John Collinge, et al., "Spongiform Encephalopathies: A Common Agent for BSE and vCJD," Nature, October 2, 1997).

The same week that Collinge’s conclusive second Nature article was published, Prusiner’s research brought him a Nobel Prize in medicine.

It was estimated that, by the fall of 1996, at least 750,000 cattle infected with mad cow disease had entered the human food chain since the start of the epidemic. You may wish to read that sentence again (R.M. Anderson, et al., "Transmission Dynamics and Epidemiology of BSE in British Cattle," Nature, August 29, 1996).

Many people will die because their government refused to tell them it was no longer safe to eat meat. Politicians frequently retire from "public service" with a sizeable amount of money in the bank. There is a reason.

The incubation period of the spongiform diseases varies in direct relation to a species’ natural life expectancy. Mice will show visible symptoms of the disease within a few months after being infected. It takes cats several years for symptoms to reveal themselves. The incubation period in humans of CJD can vary from 4 to 10 years or more.

Therefore the cases of CJD that have arisen in the first half of the 1990s could well have derived from the eating of infected beef in the early or mid-1980s, before BSE was even diagnosed.

But, obviously, the quicker a person stops eating meat now, the more likely he will avoid contracting the disease. A beef steak is not worth dying for.

At the present time, there is no treatment for CJD. If you have it, you will die from it. No drugs, surgery, or natural remedies can remove it from your body. There is no test for CJD to determine if you have it, before symptoms reveal themselves, other than a biopsy of brain material after you are dead.

To date, the U.S. Government refuses to test food animals, to see if they have the American form of BSE. This could easily be done by testing a large sampling of downer cows. They died from what appears to be BSE, but no one will test them. The government and meat industry do not want them tested and researchers are glad to avoid the task; for it would contaminate their laboratories.

Richard Lacey predicts that 200,000 (two hundred thousand!) Britishers will die each year, by the year 2015 (Rhodes, p. 222).