title: Theoretical analysis of the implication of PrP in neuronal death during transmissible subacute spongiform encephalopathies: hypothesis of a PrP oligomeric channel.
abstract: 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.
authors.affiliation: CEA/DBMS/Biophysique Moléculaire et Cellulaire, URA/CNRS520, CEA/Grenoble, 17 Rue des Martyrs, Grenoble, 38054, France. yves.chapron@wanadoo.fr
authors.forename: Y
authors.initials: Y
authors.lastname: Chapron
authors.forename: J M
authors.initials: JM
authors.lastname: Peyrin
authors.forename: S
authors.initials: S
authors.lastname: Crouzy
authors.forename: A
authors.initials: A
authors.lastname: Jaegly
authors.forename: D
authors.initials: D
authors.lastname: Dormont
mesh_terms: D000818:Animals; D017209:Apoptosis / Q000502:physiology; D006801:Humans; D007473:Ion Channels / Q000502:physiology*; D008954:Models, Biological; D009474:Neurons / Q000473:pathology*; D017096:Prion Diseases / Q000473:pathology / Q000503:physiopathology*; D011328:Prions / Q000472:pathogenicity* / Q000502:physiology; D011487:Protein Conformation
chemical_list: D007473:Ion Channels; D011328:Prions