Few conditions are more detrimental to human brains than the one popularly referred to as mad cow disease. But now theres reason to suspect that the protein which, when malformed, causes bovine spongiform encephalopathy in cows and Creutzfeldt-Jakob disease in people, might also be necessary for healthy brain function. Researchers from Whitehead Institute for Biomedical Research and Harvard Medical School/Massachusetts General Hospital have discovered that the normal form of this detrimental protein may actually help the brain create neurons, those electricity-conducting cells that make cognition possible.
"Its been difficult to understand why this prion protein, which when malformed subjects us to this horrible disease, is so abundant in our brains in the first place," says Whitehead Member Susan Lindquist, who is also a professor of biology at MIT. Along with Jeffrey Macklis of Harvard Medical School and Massachusetts General Hospital, she is co-senior author on this Proceedings of the National Academy of Sciences paper, scheduled to be published the week of February 13. "Weve known for years what happens when this protein goes wrong. Now were starting to see what its normal form does right."
For over ten years, researchers have known that a protein called PrP causes mad cow disease and its human equivalent, Creutzfeld-Jakob disease, when it forms incorrectly. 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 or even beneficial. But in mammals, it can lead to the fatal brain lesions that characterize diseases such as Creutzfeld-Jakob.
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