Could They All Be Prion Diseases?

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Science  04 Dec 2009:
Vol. 326, Issue 5958, pp. 1337-1339
DOI: 10.1126/science.326.5958.1337

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Misfolded proteins called prions are the cause of several neurodegenerative disorders in humans and other animals. A recent flurry of papers has revived interest in the idea that such mechanisms may play a role in an even wider range of neurodegenerative disorders, including two of the most dreaded scourges of old age: Alzheimer's and Parkinson's diseases. Such diseases almost certainly aren't contagious like true prion diseases are, at least in ordinary circumstances, but they may propagate through the nervous system in much the same way. Evidence from recent animal studies suggests that many of the misfolded proteins thought to play a central role in a wide range of neurodegenerative disorders can, like prions, "seed" the misfolding and aggregation of their normally folded kin. In some cases, these pathological protein clusters appear to propagate from cell to cell. Such a mechanism could help explain several puzzles—such as why some neurodegenerative disorders tend to spread from one part of the nervous system to another in a characteristic pattern, and why some researchers have found pathological protein deposits in fetal stem cells transplanted into the brains of Parkinson's patients.