Is loss of function of the prion protein the cause of prion disorders?

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 activit...

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Bibliographic Details
Published in:Trends in molecular medicine 2003-06, Vol.9 (6), p.237-243
Main Authors: Hetz, Claudio, Maundrell, Kinsey, Soto, Claudio
Format: Article
Language:English
Subjects:
Animals
Cattle
GPI-Linked Proteins
Humans
Mice
Mice, Knockout
Models, Biological
Neurons - pathology
Prion Diseases - etiology
Prion Diseases - pathology
Prions - metabolism
Prions - physiology
Protein Folding
Protein Isoforms - metabolism
Signal Transduction
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Summary: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.
ISSN:1471-4914
1471-499X
DOI:10.1016/S1471-4914(03)00069-8