NR AOGD
AU Hetz,C.A.; Maundrell,K.; Soto,C.
TI Is loss of function of the prion protein the cause of prion disorders?
QU Trends in Molecular Medicine 2003 Jun; 9(6): 237-43
PT journal article
AB Transmissible spongiform encephalopathies are fatal neurodegenerative diseases that involve misfolding of the prion protein. Recent studies have provided evidence that normal prion protein might have a physiological function in neuroprotective signaling, suggesting that loss of prion protein activity might contribute to the pathogenesis of prion disease. However, studies using knockout animals do not support the loss-of-function hypothesis and argue that prion neurodegeneration might be associated with a gain of a toxic activity by the misfolded prion protein. Thus, the mechanism of neurodegeneration in spongiform encephalopathies remains enigmatic.
MH Animals; Cattle; Human; Mice; Mice, Knockout; Models, Biological; Neurons/pathology; Prion Diseases/*etiology/pathology; Prions/metabolism/*physiology; Protein Folding; Protein Isoforms/metabolism; Signal Transduction
AD Serono Pharmaceutical Research Institute, 14 Chemin des Aulx, 1228 Plan les Ouates, Switzerland.
SP englisch
PO England