[HTML][HTML] Mammalian prion biology: one century of evolving concepts

A Aguzzi, M Polymenidou - Cell, 2004 - cell.com
A Aguzzi, M Polymenidou
Cell, 2004cell.com
Prions have been responsible for an entire century of tragic episodes. Fifty years ago, kuru
decimated the population of Papua New Guinea. Then, iatrogenic transmission of prions
caused more than 250 cases of Creutzfeldt-Jakob disease. More recently, transmission of
bovine spongiform encephalopathy to humans caused a widespread health scare. On the
other hand, the biology of prions represents a fascinating and poorly understood
phenomenon, which may account for more than just diseases and may represent a …
Abstract
Prions have been responsible for an entire century of tragic episodes. Fifty years ago, kuru decimated the population of Papua New Guinea. Then, iatrogenic transmission of prions caused more than 250 cases of Creutzfeldt-Jakob disease. More recently, transmission of bovine spongiform encephalopathy to humans caused a widespread health scare. On the other hand, the biology of prions represents a fascinating and poorly understood phenomenon, which may account for more than just diseases and may represent a fundamental mechanism of crosstalk between proteins. The two decades since Stanley Prusiner's formulation of the protein-only hypothesis have witnessed spectacular advances, and yet some of the most basic questions in prion science have remained unanswered.
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