Mad Cow Research: Small Steps, Big Questions

Jan. 22 -- Mad cow disease is so terrifying and perplexing that some researchers have begun to believe it could have alien origins.

Two astronomy and mathematics professors in England announced last month that cows in England and Wales may have picked up the disease after eating grass laced with a sprinkling of interstellar dust. The dust, the scientists proposed, fell as the Earth was bombarded by comets which hosted infectious, extraterrestrial matter.

The notion may seem outlandish (and many scientists think it is), but research shows the disease, itself, is outlandish. And its bizarre nature has stumped many efforts to find effective screening tools and treatment.

"When you're trying to design drugs, it's especially difficult when you don't fully understand the nature of the infectious agent," says Byron Caughey, a biochemist at the Rocky Mountain Laboratory branch of the National Institutes of Health.

Infectious, Warped Proteins

To defeat the disease, however, scientists must understand its nature. So researchers across the U.S. and Europe are furiously studying mad cow and its infectious agent's puzzling behavior to try and come up with treatments as well as better screening tests for the disease.

Creuztfeld-Jacob disease, one of a group of terrible animal and human diseases called Transmissible Spongiform Encephalopathies (TSE), has been recognized as a rare human scourge for decades. But a new variant of the disease emerged in Great Britain in the late 1980s which scientists believe is related to eating beef infected with BSE, or mad cow disease.

Research so far suggests mad cow disease and its cousins, which attack the brain and slowly transform it into a spongy mass of useless tissue, are caused by a most unlikely agent — a protein. These proteins, known as prions, are nearly normal, except that rather than folding into an intricate, orderly pattern, the strings of these proteins are refolded into tough and toxic deposits.

These abnormal prions infect the body by binding with normal prion proteins and triggering them to take on the corrupted structure of the rogue proteins. The body's weapons, known as protease enzymes, which chop up and dispose of unwanted proteins, are rendered useless against the transformed prion proteins.