An article published in the journal PLOS Pathogens reports a realistic computational model for the structure and mechanism of replication of prions, infectious agents responsible for mad cow disease and other neurodegenerative disorders of human and animals.
The study was carried out in the Dulbecco Telethon Laboratory of Prions & Amyloids at CIBIO, lead by Emiliano Biasini, University of Trento and involved the team led by Prof. Pietro Faccioli, a physicist from the same university and affiliated to the Italian National Institute of Nuclear Physics.
Prions are unusual infectious agents made by aberrantly folded forms of a physiological protein called the cellular prion protein, or PrPC.
These pathogens are known to replicate in absence of genetic material by recruiting normal PrPC molecules at the surface of cells and forcing them to change conformation and become infectious themselves.
The resulting accumulation of prion particles in the nervous system lies at the root of neurodegenerative conditions known as transmissible spongiform encephalopathies, including Creutzfeldt-Jakob disease, fatal familial insomnia and Gerstmann-Sträussler-Scheinker in human, but also a variety of other pathologies in mammals such as the famous mad cow disease, which in the nineties caused a large epidemics in UK and Europe and several cases of cross-species transmission to human caused by the ingestion of infected meat.
Telethon researchers revised previous models of prion structure and proposed a novel architecture consistent with recent experimental data.
This new model allowed Pietro Faccioli’s group to apply their innovative algorithms for the reliable prediction of protein conformational transitions to the prion replication mechanism.
The study has been supported by the Italian Telethon Foundation and included collaborators from the University of Santiago de Compostela (Spain) and the University of Alberta (Canada).
In the press release, available in the download box, the comments of Emiliano Biasini, Assistant Telethon Scientist and Associate Professor at the Department CIBIO, University of Trento – Giovanni Spagnolli, Ph.D. student at the Department CIBIO, University of Trento and first author of the paper – and Pietro Faccioli, Associate Professor at the Department of Physics, University of Trento and affiliated to the Italian National Institute for Nuclear Physics.
Further information could be requested to:
Ufficio stampa Fondazione Telethon – HAVAS PR Milan
thomas.balanzoni [at] havaspr.com
Ufficio stampa Università degli Studi di Trento
alessandra.saletti [at] unitn.it
INFN press office
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