Theoretical Analysis of the Implication of PrP in Neuronal Death during Transmissible Subacute Spongiform Encephalopathies: Hypothesis of a PrP Oligomeric Channel

Transmissible subacute spongiform encephalopathies (TSE) are animal and human neurodegenerative diseases. The nature of the transmissible agent remains unknown. The specific molecular marker of these diseases is the abnormal isoform of the prion protein (PrP). This protein is encoded by a cellular g...

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Bibliographic Details
Published in:Journal of theoretical biology 2000-05, Vol.204 (1), p.103-111
Main Authors: CHAPRON, YVES, PEYRIN, JEAN-MICHEL, CROUZY, SERGE, JAEGLY, ALEXANDRE, DORMONT, DOMINIQUE
Format: Article
Language:English
Subjects:
Animals
Apoptosis - physiology
Humans
Ion Channels - physiology
Models, Biological
Neurons - pathology
Prion Diseases - pathology
Prion Diseases - physiopathology
Prions - pathogenicity
Prions - physiology
Protein Conformation
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Summary:Transmissible subacute spongiform encephalopathies (TSE) are animal and human neurodegenerative diseases. The nature of the transmissible agent remains unknown. The specific molecular marker of these diseases is the abnormal isoform of the prion protein (PrP). This protein is encoded by a cellular gene and accumulates in a pathological isoform (PrPres) which is partially resistant to proteolysis. The tridimensional structure of this protein remains theoretical. F. Cohen proposed one of the most realistic models. According to this model and from molecular mechanics calculation, we suggest a PrP oligomeric ionic channel model that may be involved in TSE-induced neuronal apoptosis.
ISSN:0022-5193
1095-8541
DOI:10.1006/jtbi.2000.1091