The cellular prion protein (PrPC) has been associated with a plethora of cellular functions ranging from cell cycle to neuroprotection. Mice lacking PrPC 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 PrPC 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 PrPC plays a role in neuroprotection in KA-treated cells and mice. For this function, PrPC 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 PrPC in the KA-mediated responses in B6129 and B6.129 Prnp(0/0) mice.

Involvement of PrPC in kainate-induced excitotoxicity in several mouse strains / Carulla, P.; Llorens, F.; Matamoros Angles, A.; Aguilar Calvo, P.; Espinosa, J. C.; Gavín, R.; Ferrer, I.; Legname, Giuseppe; Torres, J. M.; del Río, J. A.. - In: SCIENTIFIC REPORTS. - ISSN 2045-2322. - 5:July(2015), pp. 11971.1-11971.15. [10.1038/srep11971]

Involvement of PrPC in kainate-induced excitotoxicity in several mouse strains

Legname, Giuseppe;
2015-01-01

Abstract

The cellular prion protein (PrPC) has been associated with a plethora of cellular functions ranging from cell cycle to neuroprotection. Mice lacking PrPC 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 PrPC 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 PrPC plays a role in neuroprotection in KA-treated cells and mice. For this function, PrPC 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 PrPC in the KA-mediated responses in B6129 and B6.129 Prnp(0/0) mice.
2015
5
July
1
15
11971
10.1038/srep11971
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4648388/
https://www.nature.com/articles/srep11971
Carulla, P.; Llorens, F.; Matamoros Angles, A.; Aguilar Calvo, P.; Espinosa, J. C.; Gavín, R.; Ferrer, I.; Legname, Giuseppe; Torres, J. M.; del Río, J. A.
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/20.500.11767/14923
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