First Glimpse at Infectious Prion Shape

The preliminary structure of the misfolded protein that causes mad cow disease and Creutzfeldt-Jakob disease looks like a coiled mattress spring.

Abby Olena, PhD

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Researchers piece together the preliminary structure of the prion that causes mad cow disease Molecular architecture of an infectious mammalian prion.IMAGE CREDIT: ESTER VAZQUEZ-FERNANDEZ, HOWARD YOUNG, HOLGER WILLE, JESUS REQUENA

Mammalian prions are notoriously difficult as structural biology subjects, given their insolubility and tendency to aggregate. Researchers have now overcome these challenges to figure out the preliminary structure of a shortened form of infectious prion (PrP^Sc), which they report today (September 8) in PLOS Pathogens.

“For the first time, we have a structure of an infectious mammalian prion,” said Giuseppe Legname of Scuola Internazionale Superiore di Studi Avanzati in Trieste, Italy, who was not involved in this study. “It’s a very important paper,” he added.

“What we have done is to obtain a very simple, very preliminary idea of what the structure of these mammalian prions are,” said study coauthor Jesús Requena of the University of Santiago de Compostela in Spain.

Requena and colleagues generated ...

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