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Sci Rep. 2015 Jul 9;5:11971. doi: 10.1038/srep11971.

Involvement of PrP(C) in kainate-induced excitotoxicity in several mouse strains.

Author information

1
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.
2
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.
3
Centro de Investigación en Sanidad Animal (CISA-INIA), Valdeolmos, Madrid, Spain.
4
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.
5
Laboratory of Prion Biology, Department of Neuroscience, Scuola Internazionale Superiore di Studi Avanzati (SISSA), Trieste, Italy.

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.

PMID:
26155834
PMCID:
PMC4648388
DOI:
10.1038/srep11971
[Indexed for MEDLINE]
Free PMC Article

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