The Bright Side of Prions

Associated with numerous neurological diseases, misfolded proteins may also play decisive roles in normal cellular functioning.

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© ALFRED PASIEKA/SCIENCE SOURCE

In Kurt Vonnegut’s Cat’s Cradle, scientists create a highly stable form of crystalline water called “ice-nine” that stays frozen even at high temperatures. Ice-nine instantly freezes any liquid water it touches. Its accidental release into nature solidifies the oceans and all contiguous bodies of water, and global catastrophe threatens our existence. Luckily for us, ice-nine is fictitious. But its biological counterpart, unfortunately, is not. The misfolded proteins known as prions are very real.

Prions are proteinaceous infectious particles, formed when normal proteins misfold and clump together. Biochemists Byron Caughey of the National Institute of Allergy and Infectious Diseases and Peter Lansbury of Brigham and Women’s Hospital were among the first to explore the analogy between Vonnegut’s ice-nine and prions in their 1995 review of scrapie, ...

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