CBS National TV Part Three on Mad Cow USA

Wednesday, January 31, 2001 5:07 PM
Subject: The Science Of Mad Cow Disease (3 of 3) & 1/31/01 CBS News,1597,268516-412,00.shtml
CBS NEWS BROADCAST, Wednesday, January 31, 2001

"The end result of this accumulation of prions in the brain are . big
holes in the brain. A swiss cheese appearance."
Dr. Stanley Prusiner, Nobel laureate

The Science Of Mad Cow Disease

* Scientists Race To Develop A Blood Test For Mysterious Prions
* Researchers Say Prions Are Linked To Brain-Wasting Disease
* Experts Assure Prions Are Not Contagious

SAN FRANCISCO, Jan. 31, 2001

AP(CBS) In the last report in a three-part series, CBS News
Correspondent Wyatt Andrews reports on the mysterious agent that causes
mad cow disease, the prion, and steps scientists are taking to develop a
test for it.
As if mad cow disease isn't frightening enough, the cause is the stuff
of science fiction. The cause is a mutant protein that kills the brain.
It's called a prion and scientists haven't seen anything like it since
Louis Pasteur found germs.

"I describe the prion as a completely new infectious particle. It's not
a virus, not a bacterium, not a fungus. Different," said Dr Stanley

Prusiner, of the University of California, San Francisco, won the Nobel
prize in medicine for discovering the prion. It's a natural protein in
mammals that can mysteriously deform itself, replicate and concentrate
in the brain.

"The end result of this accumulation of prions in the brain are these
big holes in the brain. A swiss cheese appearance," described Prusiner,
in which the brain is wasting away.

In the 1950s a disease similar to mad cow was found in Papua New Guinea.
A tribe there got a brain-wasting disease called kuru, after eating the
prion-infected brains of dead relatives in a religious ritual.
Cannibalism is the link. Mad cow, it seems, comes from healthy cows
eating the remains of infected cows in feed. Somehow, the infectious
prions jump to humans in beef.

The outbreak in Europe has launched a scientific race to find a blood
test for prions. The idea is if you can find the infection in cows, you
can prevent it in people.

"We can take all the animals that are positive, that are infected with
prions, if your test is good enough, and we can keep all these animals
out of the human food chain," said Prusiner.

Also working on a blood test is Dr. Robert Gallo at the University of
Maryland. Gallo, the same man who discovered HIV, says there's nothing
as indestructible as a prion. If it was in your meat, Gallo says, it
could not be cooked out.

Gallo is unsure about the threat from mad cow. "We have to be concerned
enough that we should be prepared. But I can't answer you that we should
be scared."

There are some reasons not to be alarmed by prion diseases. They are not
thought to be contagious person to person, and despite the rising mad
cow numbers in Europe, that disease is still very rare. However, of all
the creatures found with a prion disease - cows, sheep, elk, human
beings - not a single victim has ever recovered.

And here is the wild card. Remember that cannibal tribe? Some did not
get sick for 40 years. To scientists, that means a small, but real, risk
thousands of human carriers are incubating mad cow prions now, but don't

When asked if, in his darkest moment he thought that this is the plague
of the 21st Century, Prusiner said, "I don't need a dark moment to
wonder if that's the case, because everybody's wondering that, not just

Prusiner is certain someone will find a medicine to neutralize prions.
Right now, mad cow is hard to detect, impossible to treat, and its true
reach into human beings is unknown.

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