Prions and AmyloidsSelf-propagating protein structures in mammals, yeast and fungi

Published September 2008 16 lectures
Prof. Reed Wickner
National Institutes of Health, USA
Summary

Prions (infectious proteins) have come to particular prominence with the emergence of the Mad Cow disease epidemic and its (fortunately rare) transmission to humans in the UK. But the interest in prions as a non-nucleic acid mechanism of transmission of genetic information, and a model for the very common amyloid... read morediseases is a more enduring focus of this field. Beyond the mammalian prions, the discovery of prions in the yeast Saccharomyces cerevisiae and the filamentous fungus, Podospora anserina, have propelled the field forward, with new approaches and insights into the prion phenomenon and even potential approaches for treatment of the mammalian prion diseases.

Most prions of yeast, fungi and mammals are self-propagating amyloid forms of chromosomally encoded proteins. Studies of these infectious amyloids (prions) has revealed a great deal of information relevant to amyloids in general. In the opposite direction, detailed studies of the structures of non-prion amyloids has recently led to a better understanding of prions as well.