Molecular Mechanisms of Prion Diseases
Hermann C. Altmeppen, Berta Puig, Susanne Krasemann, Clemens Falker, Frank Dohler and Markus Glatzel
from: Prions: Current Progress in Advanced Research (Edited by: Akikazu Sakudo and Takashi Onodera). Caister Academic Press, U.K. (2013)
Prion diseases or transmissible spongiform encephalopathies are fatal neurodegenerative conditions occurring in humans and animals that may be transmitted. Experimental data and neuropathological examinations show that prions (here defined as the agent responsible for transmissible spongiform encephalopathies) consist of a self-propagating isoform of the cellular prion protein. 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 ...