What do mad cow disease and stem cell research have in common? Whitehead Institute scientists have found that the same protein that causes neurodegenerative conditions such as bovine spongiform encephalopathy (mad cow disease) is also important for helping certain adult stem cells maintain themselves.
"For years weve wondered why evolution has preserved this protein, what positive role it could possibly be playing," says Whitehead Member Susan Lindquist. Along with Whitehead Member Harvey Lodish, Lindquist is a coauthor on the paper which will published online in Proceedings of the National Academy of Sciences during the week of January 30. "With these findings, we have our first answer," she says.
For over ten years, researchers have known that a protein called PrP causes mad cow disease and its human equivalent, Creutzfeld-Jakob disease. PrP is a prion, a class of proteins that has the unusual ability to recruit other proteins to change their shape (PrP is shorthand for "prion protein."). This is significant, because a proteins form determines its function. When a prion changes shape, or "misfolds," it creates a cascade where neighboring proteins all assume that particular conformation. In some organisms, such as yeast cells, this process can be harmless, even beneficial. But in mammals, it can lead to the fatal brain lesions that characterize diseases such as Creutzfeld-Jakob.
David Cameron | EurekAlert!
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