TY  - CHAP
AU  - Audrey Ragagnin
AU  - Qili Wang
AU  - Aurélie Guillemain
AU  - Siaka Dole
AU  - 
Anne-Sophie Wilding
AU  - Valérie Demais
AU  - Cathy Royer
AU  - Anne-Marie Haeberlé
AU  - 
Nicolas Vitale
AU  - Stéphane Gasman
AU  - Nancy Grant
AU  - Yannick Bailly
ED  - Ivo Nikolaev Sirakov
Y1  - 2018-11-05
PY  - 2018
T1  - Prion Proteins and Neuronal Death in the Cerebellum
N2  - The cellular prion protein (PrPC) is expressed as a cell surface protein mainly in the central and peripheral nervous system. The discovery that prions are infectious agents changed the concept of protein synthesis in modern biology and built a bridge between the genesis of infectious and genetic diseases. This book discusses the normal function of the PrPC and its modulatory role in synaptic mechanisms. It describes the pathophysiological processes that accompany TSE - neurotoxicity, loss of anti-inflammatory protective function, neuronal death including prion-induced autophagy and apoptosis, and accumulation of PrPSc in the cytoplasm of neurons. Another aspect outlined here is that some prion diseases show strain variations that determine their development, demonstrating their key role in the development and progression of TSE.
BT  - Prions
SP  - Ch. 2
UR  - https://doi.org/10.5772/intechopen.80701
DO  - 10.5772/intechopen.80701
SN  - 978-1-78985-018-5
PB  - IntechOpen
CY  - Rijeka
Y2  - 2024-04-18
ER  -