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Misfolding the key to protein's ability to kill brain cells

COLUMBUS, Ohio Researchers may have discovered the mechanism behind how prions pieces of protein molecules can kill nerve cells in the brain and lead to some serious degenerative diseases. The key seems to lie in how one particular protein misfolds within an organelle inside the cell, transforming itself into a new agent and then poisoning the neuron in which it was made.

The finding was reported today in one of two papers published in the journal Science by researchers at Ohio State University and the Whitehead Institute for Biomedical Research at MIT. One report offers the best explanation to date of how these diseases transmissible spongiform encephalopathies, or TSE are able to destroy individual nerve cells and ultimately kill patients.

The second paper proposes an answer to a puzzle that has boggled researchers trying to understand how the non-infectious forms of these diseases initially gain a foothold. If proven true, then the new findings may have implications for new therapies against these diseases and even offer warnings concerning specific therapies for other maladies.

TSEs can attack most mammals. In humans, the diseases include Creutzfeld-Jakob Disease, Kuru, Fatal familial Insomnia, and
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Contact: Jiyan Ma
ma.131@osu.edu
614-688-0408
Ohio State University
17-Oct-2002


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