Simple Sugar (Trehalose) Curbs Huntington Disease in Mice
Date: Wed, 21 Jan 2004 13:32:48 +0100
Source: Reuters Health, 19 jan. 2004
Simple sugar curbs Huntington disease, in mice
Last Updated: 2004-01-19 15:47:42 -0400 (Reuters Health)
By Megan Rauscher
NEW YORK (Reuters Health) - Huntington
disease is an inherited condition caused by a genetic mutation that invariably leads to
dementia and death, usually in adulthood. For people in families with the disease, a
genetictest can tell if they have inherited the mutation -- but many prefer not to be
tested, because there is no cure.
Now comes a ray of hope.
In mice that develop a form of Huntington
disease, a non-toxic sugar compound called trehalose, given by mouth, significantly
extends life, according to Japanese researchers.
These results "make trehalose
promising as a therapeutic drug or lead compound" for the treatment of Huntington
disease, Dr. Nobuyuki Nukina and colleagues from the RIKEN Brain Science Institute in
Saitama write in this week's online edition of Nature Medicine.
There is considerable evidence that
clumping in the brain of an abnormal, insoluble protein called "huntingtin"
causes Huntington disease. Nukina's team found through lab experiments that a number of
sugars --disaccharides -- inhibit this aggregation.
"Trehalose has the strongest
effect," he told Reuters Health.
Mice with Huntington disease given
trehalose in drinking water had substantially fewer huntingtin protein aggregates in the
brain, less motor dysfunction, and lived significantly longer than untreated animals.
However, although trehalose appears to prevent the formation of new aggregates, it does
not seem to reverse existing formations.
"The protection of aggregation
formation is important to block the disease cascade," Nukina explained.Trehalose,
which is turned into glucose in the body, did not alter blood levels of glucose in the
animals. This is notable, the researchers say, because people with Huntington disease are
prone to develop diabetes.
"It is necessary to evaluate the
effectiveness, dose and safety of trehalose in human trials," Nukina concluded.
The researcher pointed out that trehalose
may also have potential against other neurological disorders caused by similar aggregation
of proteins such as Alzheimer disease, Parkinson disease, prion disease (such as the human
version of mad cow disease), and Lou Gehrig disease.