TY - JOUR
T1 - The cellular prion protein
T2 - Biochemistry, topology, and physiologic functions
AU - Griffoni, Cristiana
AU - Toni, Mattia
AU - Spisni, Enzo
AU - Bianco, Maria Cristina
AU - Santi, Spartaco
AU - Riccio, Massimo
AU - Tomasi, Vittorio
PY - 2003/6
Y1 - 2003/6
N2 - Studies on the transmission from man to animals of Creutzfeld-Jacob disease (CJD) led Prusiner to identify a proteinaceous infectious particle lacking nucleic acid, which was called priori. The identification of the infectious prion (PrPsc) then led to the discovery of the normal cellular counterpart (PrPc). One of the still enigmatic aspects regarding prion diseases is actually how, where, and when the transformation PrPc/PrPsc is occurring, this being due to the result of a large extent to the fact that so far most studies have been dedicated to the formation and transmission of PrPsc, whereas the understanding of physiologic roles of PrPc are in their infancy. In this review, we hope to identify the most reliable hypotheses for future experiments on PrPc. This is relevant not only for the understanding of PrPc functions but also to unravel the enigmatic nature of PrPc/PrPsc conversion.
AB - Studies on the transmission from man to animals of Creutzfeld-Jacob disease (CJD) led Prusiner to identify a proteinaceous infectious particle lacking nucleic acid, which was called priori. The identification of the infectious prion (PrPsc) then led to the discovery of the normal cellular counterpart (PrPc). One of the still enigmatic aspects regarding prion diseases is actually how, where, and when the transformation PrPc/PrPsc is occurring, this being due to the result of a large extent to the fact that so far most studies have been dedicated to the formation and transmission of PrPsc, whereas the understanding of physiologic roles of PrPc are in their infancy. In this review, we hope to identify the most reliable hypotheses for future experiments on PrPc. This is relevant not only for the understanding of PrPc functions but also to unravel the enigmatic nature of PrPc/PrPsc conversion.
KW - Caveolae
KW - Copper binding
KW - Prion protein
KW - Rafts
KW - Signal transduction
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U2 - 10.1385/CBB:38:3:287
DO - 10.1385/CBB:38:3:287
M3 - Article
C2 - 12794269
AN - SCOPUS:2142728575
VL - 38
SP - 287
EP - 304
JO - Cell Biochemistry and Biophysics
JF - Cell Biochemistry and Biophysics
SN - 1085-9195
IS - 3
ER -