NR ASUQ
AU Campana,V.; Sarnataro,D.; Zurzolo,C.
TI The highways and byways of prion protein trafficking
QU Trends in Cell Biology 2005 Feb; 15(2): 102-11
PT journal article; review; review, tutorial
AB Prions are defined as infectious agents that comprise only proteins and are responsible for transmissible spongiform encephalopathies (TSEs) - fatal neurodegenerative diseases that affect humans and other mammals and include Creutzfeldt-Jakob disease in humans, scrapie in sheep and bovine spongiform encephalopathy in cattle. Prions have been proposed to arise from the conformational conversion of the cellular prion protein PrPc to a misfolded form termed PrPsc that precipitates into aggregates and fibrils. The conversion process might be triggered by interaction of the infectious form with the cellular form or it might result from a mutation in the gene encoding PrPc. Exactly how and where in the cell the interaction and the conversion of PrPc to PrPsc occur, however, remain controversial. Recent studies have shed light on the intracellular trafficking of PrPc, the role of protein mis-sorting and the cellular factors that are thought to be required for the conformational conversion of prion proteins.
ZR 81
MH Animals; Cell Membrane/metabolism; Endoplasmic Reticulum/metabolism; Humans; Membrane Microdomains/metabolism; Models, Biological; PrPc Proteins/metabolism; PrPsc Proteins/metabolism; Prions/*metabolism; Protein Transport/physiology; Research Support, Non-U.S. Gov't; Transport Vesicles/metabolism
AD Dipartimento di Biologia e Patologia Cellulare e Molecolare, Centro di Endocrinologia ed Oncologia Sperimentale del Consiglio Nazionale delle Ricerche, Universita degli Studi di Napoli Federico II, 80131 Naples, Italy.
SP englisch
PO England