Living With Huntington Disease

As announced at the Huntington Society's Annual Conference in Halifax on 20
October, there's more important news on the research front C two
announcements from the United States move us closer still to our goal of
defeating Huntington disease.

In the 18 October edition of Nature, a team of scientists at the University
of California at Irvine announced that they had prevented cell death in the
fruit fly model of Huntington's using compounds called Ahistone deacetylase
inhibitors@ (HDAC). Histone deacetylase inhibitors are currently being
tested as chemotherapy agents for cancer, and could be moved quickly into
clinical trials for Huntington disease and other neurodegenerative
disorders.

In the 15 October 2001 edition of Nature Genetics, investigators in
Rochester have proposed a new model of cell death in HD. Until now, it has
been thought that the abnormal protein produced by the HD gene breaks down
in cells, and that damage is caused by the fragments containing the expanded
region which characterizes HD. This new study suggests that the abnormal
protein actually resists being broken down, and that it swallows up other
proteins in brain cells, including normal huntingtin protein, thereby
causing a loss of cell function and eventual cell death.

These two announcements are complementary in a very important respect,
because they illustrate remarkable progress on both the scientific and
clinical fronts. It's clear that our understanding of what goes wrong in
cells affected by Huntington disease is moving forward hand-in-hand with the
identification of new compounds which may prove to be beneficial for
affected individuals.

Between 1998 and now, the Huntington Society of Canada's financial
commitment to HD research has doubled. Through its NAVIGATOR Coalition
programme, the Society is a leader of the global campaign to discover a cure
for Huntington disease.
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