title: Involvement of PrP(C) in kainate-induced excitotoxicity in several mouse strains.
abstract: The cellular prion protein (PrP(C)) has been associated with a plethora of cellular functions ranging from cell cycle to neuroprotection. Mice lacking PrP(C) show an increased susceptibility to epileptic seizures; the protein, then, is neuroprotective. However, lack of experimental reproducibility has led to considering the possibility that other factors besides PrP(C) deletion, such as the genetic background of mice or the presence of so-called "Prnp flanking genes", might contribute to the reported susceptibility. Here, we performed a comparative analysis of seizure-susceptibility using characterized Prnp(+/+) and Prnp(0/0) mice of B6129, B6.129, 129/Ola or FVB/N genetic backgrounds. Our study indicates that PrP(C) plays a role in neuroprotection in KA-treated cells and mice. For this function, PrP(C) should contain the aa32-93 region and needs to be linked to the membrane. In addition, some unidentified "Prnp-flanking genes" play a role parallel to PrP(C) in the KA-mediated responses in B6129 and B6.129 Prnp(0/0) mice.
authors.affiliation: 1] Molecular and Cellular Neurobiotechnology, Institute of Bioengineering of Catalonia (IBEC), Parc Científic de Barcelona, Barcelona, Spain [2] Department of Cell Biology, Universitat de Barcelona, Barcelona, Spain [3] Centro de Investigación Biomédica en Red sobre Enfermedades Neurodegenerativas (CIBERNED), Barcelona, Spain.
authors.forename: Patricia
authors.initials: P
authors.lastname: Carulla
authors.affiliation: 1] Molecular and Cellular Neurobiotechnology, Institute of Bioengineering of Catalonia (IBEC), Parc Científic de Barcelona, Barcelona, Spain [2] Department of Cell Biology, Universitat de Barcelona, Barcelona, Spain [3] Centro de Investigación Biomédica en Red sobre Enfermedades Neurodegenerativas (CIBERNED), Barcelona, Spain [4] German Center for Neurodegenerative Diseases (DZNE), Robert-Koch Str. 40, 37075, Göttingen, Germany.
authors.forename: Franc
authors.initials: F
authors.lastname: Llorens
authors.affiliation: 1] Molecular and Cellular Neurobiotechnology, Institute of Bioengineering of Catalonia (IBEC), Parc Científic de Barcelona, Barcelona, Spain [2] Department of Cell Biology, Universitat de Barcelona, Barcelona, Spain [3] Centro de Investigación Biomédica en Red sobre Enfermedades Neurodegenerativas (CIBERNED), Barcelona, Spain.
authors.forename: Andreu
authors.initials: A
authors.lastname: Matamoros-Angles
authors.affiliation: Centro de Investigación en Sanidad Animal (CISA-INIA), Valdeolmos, Madrid, Spain.
authors.forename: Patricia
authors.initials: P
authors.lastname: Aguilar-Calvo
authors.affiliation: Centro de Investigación en Sanidad Animal (CISA-INIA), Valdeolmos, Madrid, Spain.
authors.forename: Juan Carlos
authors.initials: JC
authors.lastname: Espinosa
authors.affiliation: 1] Molecular and Cellular Neurobiotechnology, Institute of Bioengineering of Catalonia (IBEC), Parc Científic de Barcelona, Barcelona, Spain [2] Department of Cell Biology, Universitat de Barcelona, Barcelona, Spain [3] Centro de Investigación Biomédica en Red sobre Enfermedades Neurodegenerativas (CIBERNED), Barcelona, Spain.
authors.forename: Rosalina
authors.initials: R
authors.lastname: Gavín
authors.affiliation: 1] Institut de Neuropatologia, IDIBELL-Hospital Universitari de Bellvitge, Hospitalet de Llobregat, Barcelona, Spain [2] Centro de Investigación Biomédica en Red de Enfermedades Neurodegenerativas (CIBERNED), Barcelona, Spain.
authors.forename: Isidre
authors.initials: I
authors.lastname: Ferrer
authors.affiliation: Laboratory of Prion Biology, Department of Neuroscience, Scuola Internazionale Superiore di Studi Avanzati (SISSA), Trieste, Italy.
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Legname
authors.affiliation: Centro de Investigación en Sanidad Animal (CISA-INIA), Valdeolmos, Madrid, Spain.
authors.forename: Juan Maria
authors.initials: JM
authors.lastname: Torres
authors.affiliation: 1] Molecular and Cellular Neurobiotechnology, Institute of Bioengineering of Catalonia (IBEC), Parc Científic de Barcelona, Barcelona, Spain [2] Department of Cell Biology, Universitat de Barcelona, Barcelona, Spain [3] Centro de Investigación Biomédica en Red sobre Enfermedades Neurodegenerativas (CIBERNED), Barcelona, Spain.
authors.forename: José A
authors.initials: JA
authors.lastname: del Río
mesh_terms: D000818:Animals; D015415:Biomarkers; D016923:Cell Death; D002460:Cell Line; D016207:Cytokines / Q000378:metabolism; D004195:Disease Models, Animal; D015870:Gene Expression; D020022:Genetic Predisposition to Disease; D005911:Gliosis / Q000209:etiology / Q000378:metabolism / Q000473:pathology; D006624:Hippocampus / Q000378:metabolism / Q000473:pathology; D018836:Inflammation Mediators / Q000378:metabolism; D007608:Kainic Acid / Q000633:toxicity*; D051379:Mice; D018345:Mice, Knockout; D018621:PrPC Proteins / Q000235:genetics*; D012640:Seizures / Q000209:etiology / Q000378:metabolism / Q000473:pathology; D014162:Transfection
chemical_list: D015415:Biomarkers; D016207:Cytokines; D018836:Inflammation Mediators; D018621:PrPC Proteins; D007608:Kainic Acid