Solving The Mystery Of Huntington's Disease

Published: Jul 26, 2004

Researchers believe they've found a link between competing theories regarding how Huntington's disease kills brain cells, according to a report in the online edition of the Annals of Neurology. Huntington's is an inherited, degenerative brain disease marked by movement abnormalities and psychiatric symptoms such as depression and mood swings. The disease is caused by mutations in a single gene that leads to an abnormal form of the protein called huntingtin, which accumulates in toxic deposits inside nerve cells. Why mutant huntingtin accumulates and how it might damage brain cells has been a continuing point of contention. One theory holds that a breakdown in the normal cellular cleansing process is responsible for the buildup of the bad proteins. But researchers have contended that other processes might be causing damage to Huntington's patients beyond the huntingtin buildup, including a scarcity of molecules called neurotrophins that nourish brain cells as well as problems with mitochondria, the "power plants" that produce energy for cells. Scientists at Harvard University and McLean Hospital found evidence that all may be correct -- that the mutant huntingtin protein may have several negative effects on brain cells. This may mean that therapeutic strategies will have to take the form of combinations of drugs that address the different processes, the researchers said.

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