Simple Sugar Curbs Huntington Disease, in Mice

January 20, 2004

Simple Sugar Curbs Huntington Disease, in MiceJanuary 19, 2004 02:25:30 PM PST ,

Reuters 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 genetic test 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.

SOURCE: Nature Medicine, online edition January 18, 2004.

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