Fatal brain disease holds clues to dementia

Scientists at Melbourne's Howard Florey Institute have uncovered a clue about the causes of dementia in Huntington's disease, one of the disease's symptoms, by showing that mice susceptible to Huntington's disease have problems with learning and memory before the diseases' typical movement problems appear.

The Florey scientists also discovered that in Huntington's diseased brains, information processing between neurons is disrupted, but the neurons do not die, which means the brain may respond to new anti-dementia drugs that can restore memory.

Huntington's disease is an incurable, inherited disorder of the nervous system that affects specific brain regions and inevitably leads to death. Symptoms range from disrupted control of movements and thought processes, and emotional problems. These include: jerky arm or leg movements; difficulties with speech, swallowing, concentration, memory and learning; and depression and personality changes.

Huntington's disease is caused by a mutation in a single gene. When this defective gene is passed from parent to child, 50 percent of the offspring will inherit the disorder, which can be detected by genetic testing.

Research leader Dr Anthony Hannan said his team's investigations were significant as they could lead to the development of memory restoring drugs designed especially for people with Huntington's disease.

"We have demonstrated the linkage from molecule to cell to learning and memory, and can relate this to a particular area of the brain," Dr Hannan said.

"Our work shows that the defective huntingtin gene disrupts how large groups of neurons 'talk' to one another and adjust their wiring in response to stimulation from the environment."

"Now we can better understand how a genetic change affects wiring of neurons and relate that to changes in learning, memory and behaviour in Huntington's disease."

"By better understanding the disease mechanism, we can work

Contact: Merrin Rafferty
Research Australia

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