TY - JOUR
T1 - The physiological functions of prion protein
AU - Wechselberger, Christian
AU - Wurm, Susanne
AU - Pfarr, Werner
AU - Höglinger, Otmar
PY - 2002
Y1 - 2002
N2 - Prion proteins are mentioned predominantly as unprecedented infectious pathogens in the context of transmissible spongiform encephalopathies. Since prions are devoid of nucleic acids, disease transmission must be mediated by an entirely novel mechanism. The general accepted theory proposes the conversion of cellular prion protein (PrPC) into the pathological isoform solely through conformational changes. This process favors the development of insoluble protein aggregates in the central nervous system typical for prion diseases. However, progress to elucidate the physiological functions of PrPC is still slow besides recent indications of a multifaceted network, in which PrPC seems to play a fundamental role. Possible contributions of interrupted or disturbed physiological signaling events due to the pathological prion protein isoform are presented in terms of recent findings.
AB - Prion proteins are mentioned predominantly as unprecedented infectious pathogens in the context of transmissible spongiform encephalopathies. Since prions are devoid of nucleic acids, disease transmission must be mediated by an entirely novel mechanism. The general accepted theory proposes the conversion of cellular prion protein (PrPC) into the pathological isoform solely through conformational changes. This process favors the development of insoluble protein aggregates in the central nervous system typical for prion diseases. However, progress to elucidate the physiological functions of PrPC is still slow besides recent indications of a multifaceted network, in which PrPC seems to play a fundamental role. Possible contributions of interrupted or disturbed physiological signaling events due to the pathological prion protein isoform are presented in terms of recent findings.
KW - Cellular prion protein
KW - Interacting proteins
KW - Pathological isoform
KW - Physiological functions
KW - Signal transduction cascades
KW - Prions/physiology
KW - Humans
KW - Mice, Knockout
KW - Signal Transduction/physiology
KW - Animals
KW - Protein Isoforms
KW - Mice
KW - Prion Diseases/metabolism
UR - http://www.scopus.com/inward/record.url?scp=0036440530&partnerID=8YFLogxK
U2 - 10.1006/excr.2002.5655
DO - 10.1006/excr.2002.5655
M3 - Article
C2 - 12441124
AN - SCOPUS:0036440530
SN - 0014-4827
VL - 281
SP - 1
EP - 8
JO - Experimental Cell Research
JF - Experimental Cell Research
IS - 1
ER -