An innovative microscope study described in the 14 October 2004 issue of Nature (Vol. 431, No. 7010, see pp. 805-810, and News & Views) has answered a long-standing question about the fatal neurodegenerative disorder Huntington's disease. Researchers have discovered that the tiny clumps of abnormal protein found in diseased nerve cells actually help to boost the cells' chances of survival.
Steven Finkbeiner and colleagues dosed cultured brain cells with the abnormal version of the protein huntingtin, which causes the disease. They then monitored them using an automated microscope that returns time and again to the same individual cell, even if it dies.
Cells were more likely to die if given a large dose of mutant protein. But this risk of death was reduced if the protein formed clumps called inclusion bodies. So it seems that these clumps protect against the protein's toxic effects by reducing the amount of spread throughout the cell - some experts had previously suggested that it is the clumps themselves that do the damage.
This approach potentially offers a way to work out whether the protein clumps found in other diseases such as Alzheimer's are harmful, beneficial or merely incidental, says Harry T. Orr in an accompanying News and Views article.
Steven Finkbeiner (University of California, San Francisco, CA, USA)
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Harry T. Orr (University of Minnesota, Minneapolis, MN, USA)
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(C) Nature press release.
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