NR AEDO
AU Fournier,J.G.
TI Nonneuronal cellular prion protein
QU International Review of Cytology 2001; 208: 121-60
PT journal article; review; review, academic
AB The normal cellular prion protein (PrPc) is a membrane sialoglycoprotein of unknown function having the unique property of adopting an abnormal tertiary conformation. The pathological conformer PrPsc would be the agent of transmissible spongiform encephalopathies or prion diseases. They include scrapie and bovine spongiform encephalopathy in animals and Creutzfeldt-Jakob disease in humans. The conversion of PrPc into PrPsc in the brain governs the clinical phenotype of the disease. However, the three-dimensional structure change of PrPc can also take place outside the central nervous system, in nonneuronal cells particularly of lymphoid tissue where the agent replicates. In natural infection, PrPc in nonneuronal cells of peripheral extracerebral organs may play a key role as the receptor required to enable the entry of the infectious agent into the host. In the present review we have undertaken a first evaluation of compelling data concerning the PrPc-expressing cells of nonneuronal origin present in cerebral and extracerebral tissues. The analysis of tissue, cellular, and subcellular localization of PrPc may help us better understand the biological function of PrPc and provide some information on physiopathological processes underlying prion diseases.
ZR 200
MH Animal; Central Nervous System/cytology/metabolism; Gene Expression Regulation/physiology; Human; Neurons/cytology/metabolism; PrPc Proteins/genetics/*metabolism; Prion Diseases/genetics/*metabolism/physiopathology; Reticuloendothelial System/cytology/metabolism; Support, Non-U.S. Gov't
AD Service de Neurovirologie, CEA-DSV/DRM, Fontenay aux Roses, France.
SP englisch
PO USA