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Discovery May Spur Huntington's, Parkinson's Research

A clumped protein once thought harmful may actually protect the brain, experts say

By Ed Edelson
HealthDay Reporter


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TUESDAY, March 7 (HealthDay News) -- Experts have long thought that clumps of misfolded proteins in the brains of Huntington's disease patients were a sign or cause of damage.

However, new research suggests they might actually help the brain defend itself against the debilitating illness.

Text Continues Below



A compound that boosted the formation of these "inclusions," as the clumps are called, also revved up a molecular mechanism that rids cells of abnormal proteins.

What's more, laboratory tests that focused on Parkinson's disease -- where abnormal clumping also occurs -- got the same results.

Researchers believe the finding might open a new approach to treating both of these devastating neurodegenerative conditions, said lead researcher Ruth A. Bodner, a postdoctoral fellow at the Massachusetts Institute of Technology.

Her team published its findings in this week's early online edition of Proceedings of the National Academy of Sciences.

Inclusions found in both Huntington's and Parkinson's are clearly capable of doing damage, Bodner stressed. "But, there are different conformations that the proteins can take," she added. "They can be soluble [and] they can be in the form of large inclusions, and there is increasing evidence that the large inclusions might be protective."

Just how clumped proteins help shield brain cells remains unclear. According to Bodner, it could be that having these abnormal proteins arranged in a clump hems in their toxicity. At any rate, a protein identified through a simple screening test and called B2 increased clumping and boosted the functioning of proteasomes -- molecular systems that rid the cells of bad proteins.

The search for effective treatments for Huntington's disease has typically focused on ways to prevent inclusions from forming, Bodner said. But now, "we think there is room for a different approach," she said.

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Copyright © 2006 ScoutNews LLC. All rights reserved.
Last updated 3/7/2006

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SOURCES: Ruth A. Bodner, postdoctoral fellow, Massachusetts Instutute of Technology, Cambridge; Steve Finkbeiner, M.D., Ph.D, assistant investigator, University of California, San Francisco, Gladstone Institute of Neurological Disease; March 6-10, 2006, Proceedings of the National Academy of Sciences, online


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