| Disease Background | ||
| Description of Disease | ||
| Who is at Risk? | ||
| National Cancer Institute Dictionary | ||
| Our Research | ||
| Overview of Hutchinson Center Research | ||
Research Highlights
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| Relevant Articles | ||||
| Hutchinson Center Publications | ||||
A single abnormal gene passed on by one parent to a child causes Huntington disease, an illness in which the brain's nerve-cell function is disrupted. This leads to a progressive destruction of physical, intellectual and emotional abilities and inevitably to death. The abnormal gene causes a nervous-system condition that causes jerky movements, severe emotional disturbance and mental decline. Symptoms usually strike in mid-life, in the 30s or 40s, although it also can attack children and the elderly. Consequently, part of the devastation of the disease is that by the time a person is diagnosed, most victims have started families. The disease ultimately ends in death after 10 to 25 years. There is no known treatment to halt the progression of Huntington disease.
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Anyone who has a parent with Huntington disease has a 50 percent chance that they inherited the defective gene that causes the condition. Everyone who inherits the defective gene will develop the disease. A genetic test can determine who is a carrier of the abnormal Huntington gene. In the United States, there are about 10 cases of Huntington disease for every 100,000 people — about 30,000 people in all, with another 150,000 people at risk of inheriting the disease.
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Overview of Hutchinson Center Research
How does a defect in a single gene cause a disease with such profound effects? Hutchinson Center researchers believe that answering this question is the key to developing treatments that can prevent the symptoms of Huntington disease. Our research focuses identifying the differences between brain and muscle tissue in healthy people and those with Huntington disease. These differences may serve as targets for new treatments that could stop the disease from progressing or may help doctors determine the effectiveness of new treatments before serious symptoms begin.
Based on these studies, the investigators plan to test candidate drugs in mouse models of Huntington disease. The goal is to identify drugs that may hold promise for future testing in humans.
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Spend time in San Luis, Venezuela, and something extraordinary becomes apparent. A large number of people weave down the streets, lurching and jerking with each step. In a cruel twist of heredity, one out of four people in San Luis has Huntington disease.
Like a genetic time bomb, one abnormal gene in Huntington victims disrupts the brain's nerve functions, slowly degrading physical, intellectual and emotional capability and leading inevitably to death.
Dr. Jim Olson, a Hutchinson Center researcher, first worked on Huntington nearly 20 years ago as a graduate student. He vowed then that if he could do something to help the people of San Luis, he would. Today Olson leads a group of laboratory researchers from around the nation who are using a method called DNA-array technology to understand the function of the Huntington gene as well as others involved in the development of Huntington disease and related diseases. This group is called the Hereditary Disease Array Group (HDAG).
This multi-institutional collaborative study has made rapid progress in identifying the group of genes that are immediately affected by the abnormal Huntington gene. They also have seen that the pattern of genetic changes that occurs in Huntington disease is similar to that found in other diseases that cause the nervous system to degenerate, and that degeneration occurs more rapidly with age.
As a result of the new information generated by the HDAG, researchers hope that new drugs will be developed that could be tested in human clinical trials within the next few years.
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