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Trends Biochem Sci. 2011 Mar;36(3):151-8. doi: 10.1016/j.tibs.2010.11.001. Epub 2010 Dec 3.

Prion hypothesis: the end of the controversy?

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Mitchell Center for Alzheimer's Disease and Related Brain Disorders, Department of Neurology, The University of Texas Medical school at Houston, 6431 Fannin St, Houston, TX 77030, USA.


Forty-three years have passed since it was first proposed that a protein could be the sole component of the infectious agent responsible for the enigmatic prion diseases. Many discoveries have strongly supported the prion hypothesis, but only recently has this once heretical hypothesis been widely accepted by the scientific community. In the past 3 years, researchers have achieved the 'Holy Grail' demonstration that infectious material can be generated in vitro using completely defined components. These breakthroughs have proven that a misfolded protein is the active component of the infectious agent, and that propagation of the disease and its unique features depend on the self-replication of the infectious folding of the prion protein. In spite of these important discoveries, it remains unclear whether another molecule besides the misfolded prion protein might be an essential element of the infectious agent. Future research promises to reveal many more intriguing features about the rogue prions.

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