New Understanding of Huntington’s Disease Could Unlock Cure

June 5, 2009

(ChattahBox)—Researchers from John Hopkins University have unlocked the key to what causes Huntington’s disease, a devastating genetic disorder that destroys brain cells that control movement. This new discovery may one day lead to the development of a drug to treat the disease, which affects nearly 30,000 people every year in the United States.

The John Hopkins scientists discovered a protein called “rhes” that plays a key role in causing the cell death of the brain.

Previous studies have shown that patients with Huntington’s disease are born with a genetic defect that causes a mutation of a protein, named “huntingtin,” which is found throughout a patient’s body.

However, scientists have long been puzzled by the fact that the protein only kills cells in the corpus striatum section of the brain, which controls movement and causes little damage to other cells.

This new study, focused on the protein, rhes known to only exist in the corpus striatum area of the brain. The researchers found that the protein rhes interacts in a special way with the mutant huntingtin proteins, causing cell death. This discovery can now form the basis for further research to understand the exact mechanism of the rhes protein.

The researchers tested the rhes protein in both mouse and human cells and found that when exposed to the mutated “huntingtin” genes it caused cell death, killing off half of the cells within 48 hours.

The scientists are now testing to see if the removal of rhes from mice with Huntington’s disease can slow or stop brain cells from dying. This research has great promise for the development of future drug therapies targeting the rhes protein.

This groundbreaking study is available in the June 5 issue of Science.



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