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May 19, 1999

Doctors Slow Huntington's in Mice

By DAVID KINNEY Associated Press Writer

Scientists have found a way to delay the onset of Huntington's disease
and slow its progress in mice by targeting an enzyme that is believed to
trigger cell death.

The finding, published in Thursday's issue of the journal Nature, is a
promising lead for drug researchers working to extend the lives of
Huntington's sufferers.

``I think this is important and exciting. This is the first
demonstration of a drug that can slow the progress of HD in an animal
model,'' said Dr. Christopher Ross, a Johns Hopkins Medical School
professor and a scientific adviser to the Huntington's Disease Society
of America. ``It says this goal of slowing progress probably can be
accomplished.''

Huntington's has been linked to enzymes called caspases, which cause
brain cells to die slowly.

In the new study, scientists from Harvard University's Brigham and
Women's Hospital blocked a particular caspase in mice that had been
genetically engineered to contract the disease. These mice developed
Huntington's symptoms
about 10 percent later in life and lived about 20 percent longer.

Caspase-1 apparently plays a role in the cell death that marks
Huntington's, which usually kills sufferers within 15 to 20 years. How
the enzyme works is unclear, but scientists think that it slices up the
so-called huntingtin gene, and that the fragments then kill the cell.

The Harvard researchers slowed that process by breeding mice with a
mutant protein that counteracts caspase-1.

The mice showed Huntington's symptoms - walking trouble, weight loss and
seizures - after 84 days, on average, compared with 77 days in the other
mice. The mice with the caspase blocker lived an average of 121 days,
compared with 101 days for the other mice.

``If one drug can do this, hopefully, in the future, you can find
others,'' said one of the researchers, Dr. Robert M. Friedlander.

Huntington's, which killed folk singer Woody Guthrie, afflicts 30,000
Americans.

What is exciting, Friedlander said, is that the same mechanism at work
in this study may be applied to research on brain injuries and illnesses
such as Lou Gehrig's disease and PARKINSON'S.

``Caspase inhibition may turn out to be the magic bullet against
neurodegenerative disease,'' University of Munich biochemist Christian
Haass said in an accompanying editorial.

Copyright © 1999 The Associated Press.
--
Judith Richards, London, Ontario, Canada
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