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Biology 2016, 5(1), 2; doi:10.3390/biology5010002

Disease Transmission by Misfolded Prion-Protein Isoforms, Prion-Like Amyloids, Functional Amyloids and the Central Dogma

ZBS6-Proteomics and Spectroscopy, Robert Koch-Institute, Seestrasse 10, 13353 Berlin, Germany
Academic Editor: Chris O'Callaghan
Received: 16 July 2015 / Revised: 22 October 2015 / Accepted: 28 December 2015 / Published: 4 January 2016
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Abstract

In 1982, the term “prions” (proteinaceous infectious particles) was coined to specify a new principle of infection. A misfolded isoform of a cellular protein has been described as the causative agent of a fatal neurodegenerative disease. At the beginning of prion research scientists assumed that the infectious agent causing transmissible spongiform encephalopathy (TSE) was a virus, but some unconventional properties of these pathogens were difficult to bring in line with the prevailing viral model. The discovery that prions (obviously devoid of any coding nucleic acid) can store and transmit information similarly to DNA was initially even denoted as being “heretical” but is nowadays mainly accepted by the scientific community. This review describes, from a historical point of view, how the “protein-only hypothesis” expands the Central Dogma. Definition of both, the prion principle and the Central Dogma, have been essential steps to understand information storage and transfer within and among cells and organisms. Furthermore, the current understanding of the infectivity of prion-proteins after misfolding is summarized succinctly. Finally, prion-like amyloids and functional amyloids, as found in yeast and bacteria, will be discussed. View Full-Text
Keywords: prion; Central Dogma; protein misfolding; amyloid; prion-like amyloids; functional amyloids prion; Central Dogma; protein misfolding; amyloid; prion-like amyloids; functional amyloids
This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. (CC BY 4.0).

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MDPI and ACS Style

Daus, M.L. Disease Transmission by Misfolded Prion-Protein Isoforms, Prion-Like Amyloids, Functional Amyloids and the Central Dogma. Biology 2016, 5, 2.

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