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The Prion Heretic

Science  27 May 2011:
Vol. 332, Issue 6033, pp. 1024-1027
DOI: 10.1126/science.332.6033.1024

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For 30 years, Laura Manuelidis of Yale School of Medicine has rejected the dominant theory that misfolded proteins called prions are the infectious agents behind a cluster of rare, transmissible, and fatal brain diseases called transmissible spongiform encephalopathies. When first proposed by Stanley Prusiner, the theory was widely dismissed as bizarre. Biology then held that infectious disease was caused by organisms built from DNA, RNA, or both, like viruses and bacteria—something containing a nucleic acid sequence that can replicate and spread through a cell. Proteins lack these sequences. But today, Prusiner's view dominates. Manuelidis regards the protein not as the cause of infection but as a pathological reaction to it and believes that mad cow disease and others like it are triggered by viruses—although no one has found them. She's not the only prion doubter, but her voice is by far the loudest. In part that's because as a tenured professor she's safe. Her unpopularity among top prion scientists leaves her unfazed.