Molecular Mechanisms of Prion Diseases
Hermann C. Altmeppen, Berta Puig, Susanne Krasemann and Markus Glatzel
from: Prions: Current Progress in Advanced Research (Second Edition) (Edited by: Akikazu Sakudo and Takashi Onodera). Caister Academic Press, U.K. (2019) Pages: 55-70.
Prion diseases or transmissible spongiform encephalopathies (TSE) are transmissible, fatal neurodegenerative conditions occurring in humans and animals. Experimental data and neuropathological examination show that prions (here defined as the agent responsible for TSEs) consist of a self-propagating isoform (PrPSc) of the cellular prion protein (PrPC). Nucleic acids are not required for propagation of prions. In the last years a number of questions regarding the mechanism of prion propagation and neurotoxicity, as well as the spread of prions to and within the brain have been answered, yet essential pieces of information regarding the execution of cell death and cell-to-cell spread of prions remain to be elucidated read more ...