NR AHEC
AU Lehmann,S.; Milhavet,O.; Mange,A.
TI Trafficking of the cellular isoform of the prion protein
QU Biomedicine and Pharmacotherapy 1999; 53(1): 39-46
PT journal article; review; review, tutorial
AB Transmissible spongiform encephalopathies form a group of fatal neurodegenerative disorders that have the unique property of being infectious, sporadic or genetic in origin. Although the nature of the responsible agent of these diseases is uncertain, it is clear that a protein called PrPsc has a central role in their pathology. PrPsc is a conformational variant of a normal protein called PrPc. Understanding the transition from PrPc to PrPsc is a major issue in the field. In this article, we will review what is known about the cell biology of PrPc, the understanding of which is crucial considering that trafficking of this molecule governs generation of PrPsc.
ZR 47
MH Animal; Endoplasmic Reticulum/chemistry/ultrastructure; Human; Isomerism; PrPc Proteins/*chemistry/metabolism
AD IGH du CNRS, Montpellier, France.
SP englisch
PO Frankreich
OR Prion-Krankheiten L