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Mad Cow Treatments Studied - 2001-08-19

U.S. researchers are investigating treatments for the so-called Mad Cow disease in both animals and humans. One therapy would offer a cure for the lethal disease in cows, the other would treat a variant of the fatal illness in humans, which is contracted by eating infected beef.

Mad Cow disease is technically known as bovine spongiform encephalopathy (BSE). In humans it is known as a form of Creutzfeldt-Jacob disease.

In 1986, veterinarians began identifying BSE infected cows. As of 2000, 180,000 cattle with BSE have been confirmed in Great Britain and at least 1,400 cases of Mad Cow disease have been found elsewhere in Europe. No infected cows have been found with the neurological illness in the United States.

Scientists discovered that cows became "mad" after they were infected with a mutated prion protein. Anthony Williamson, a professor of immunology at the Scripps Institute in La Jolla, California, explained that prions exist in many different tissue types in healthy individuals and animals. "But it has any ugly cousin that is basically the same protein that is folded into the same shape. And this different shape is a pathogenic form of the same protein, a rogue form of the protein. And the rogue form of the protein is able to interact with the normal form of the protein and able to cause conversion of the normal form into the abnormal form. And over the course of the disease, you get an accumulation of this abnormal form in the brain of infected individuals. And this leads to disease," he said.

Using a model of Creutzfeldt-Jacob disease in mice, Professor Williamson and colleagues developed an antibody, or immune protein, to fight mutated prions in the laboratory. "The cells we are using for experiments have an ongoing prion infection. And these cells are creating new infectious material all the time," he said.

"When we add the antibody, we find that the production of new material is halted very dramatically and then existing material is removed by the cells themselves. So, the antibody is allowing the cells to remove the infection, because the antibody is preventing the formation of any new infectious prion material."

Discovery of the antibody was reported in the British journal Nature.

The Scripps Institute's Anthony Williamson envisions using the antibody on infected cows and other animals to block the disease that appears to make them lose their minds before they die. He would also like to see a blood test to detect the disease in animals and humans.

Today, 106 people around the world, mostly in Great Britain, have shown signs of the rare neurological sickness. Researchers have surmised that they become ill by eating infected beef. Experts have named the illness "variant Creutzfeldt-Jacob disease," or "variant CJD". Traditional CJD usually strikes much older individuals.

In the current issue of Proceedings of the National Academy of Sciences, researchers at the University of California in San Francisco describe using two existing drugs to treat two patients with variant CJD. Carsten Korth is leading the study of a malaria drug and a schizophrenia drug to help people with variant CJD. These medications were chosen because they are known to enter the brain.

"We have not tried these drugs in patients, so we do not know how they will act," said Dr. Korth. "There is a possibility they will help. But there is also a possibility that they will not help at all because our results are still in the test tube."

But England's Mail on Sunday is reporting 20-year-old Rachel Forber, who is one of the two patients treated in San Francisco, is again walking and talking; nothing her father calls short of a "miracle."