Threshold for epileptiform activity is elevated in prion knockout mice

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Threshold for epileptiform activity is elevated in prion knockout mice. / Ratté, S.; Vreugdenhil, Martin; Boult, J. K. R.; Patel, A.; Asante, E. A.; Collinge, J.; Jefferys, John.

In: Neuroscience, Vol. 179, 14.04.2011, p. 56-61.

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Ratté, S. ; Vreugdenhil, Martin ; Boult, J. K. R. ; Patel, A. ; Asante, E. A. ; Collinge, J. ; Jefferys, John. / Threshold for epileptiform activity is elevated in prion knockout mice. In: Neuroscience. 2011 ; Vol. 179. pp. 56-61.

Bibtex

@article{19ada10fe6a649cf8a24ac0fc72a4faf,
title = "Threshold for epileptiform activity is elevated in prion knockout mice",
abstract = "Prion protein (PrP) is abundant in the nervous system, but its role remains uncertain. Prion diseases depend on an aggregation of the protein that is likely to interfere with its normal function. Loss of function does not in itself cause neurodegeneration, but whether it contributes to the clinical features of the disease remains an open question. Patients with classical Creutzfeldt-Jakob disease (CJD) have a higher than expected incidence of epilepsy. To study the mechanisms by which loss of PrP function may underlie changes in vulnerability to epilepsy in disease, we used several acute epilepsy models: we applied a variety of convulsant treatments (zero-magnesium, bicuculline, and pentylenetetrazol) to slices in vitro from PrP knockout (Prnp0/0) and control mice. In all three epilepsy models, we found that longer delays and/or higher concentrations of convulsants were necessary to generate spontaneous epileptiform activity in Prnp0/0 mice. These results together indicate an increased seizure threshold in Prnp0/0 mice, suggesting that loss of PrP function cannot explain a predisposition to seizures initiation in CJD.",
keywords = "prion, epilepsy, hippocampus, pentylenetetrazol, bicuculline, zero-magnesium",
author = "S. Ratt{\'e} and Martin Vreugdenhil and Boult, {J. K. R.} and A. Patel and Asante, {E. A.} and J. Collinge and John Jefferys",
year = "2011",
month = apr,
day = "14",
doi = "10.1016/j.neuroscience.2011.01.053",
language = "English",
volume = "179",
pages = "56--61",
journal = "Neuroscience",
issn = "0306-4522",
publisher = "Elsevier",

}

RIS

TY - JOUR

T1 - Threshold for epileptiform activity is elevated in prion knockout mice

AU - Ratté, S.

AU - Vreugdenhil, Martin

AU - Boult, J. K. R.

AU - Patel, A.

AU - Asante, E. A.

AU - Collinge, J.

AU - Jefferys, John

PY - 2011/4/14

Y1 - 2011/4/14

N2 - Prion protein (PrP) is abundant in the nervous system, but its role remains uncertain. Prion diseases depend on an aggregation of the protein that is likely to interfere with its normal function. Loss of function does not in itself cause neurodegeneration, but whether it contributes to the clinical features of the disease remains an open question. Patients with classical Creutzfeldt-Jakob disease (CJD) have a higher than expected incidence of epilepsy. To study the mechanisms by which loss of PrP function may underlie changes in vulnerability to epilepsy in disease, we used several acute epilepsy models: we applied a variety of convulsant treatments (zero-magnesium, bicuculline, and pentylenetetrazol) to slices in vitro from PrP knockout (Prnp0/0) and control mice. In all three epilepsy models, we found that longer delays and/or higher concentrations of convulsants were necessary to generate spontaneous epileptiform activity in Prnp0/0 mice. These results together indicate an increased seizure threshold in Prnp0/0 mice, suggesting that loss of PrP function cannot explain a predisposition to seizures initiation in CJD.

AB - Prion protein (PrP) is abundant in the nervous system, but its role remains uncertain. Prion diseases depend on an aggregation of the protein that is likely to interfere with its normal function. Loss of function does not in itself cause neurodegeneration, but whether it contributes to the clinical features of the disease remains an open question. Patients with classical Creutzfeldt-Jakob disease (CJD) have a higher than expected incidence of epilepsy. To study the mechanisms by which loss of PrP function may underlie changes in vulnerability to epilepsy in disease, we used several acute epilepsy models: we applied a variety of convulsant treatments (zero-magnesium, bicuculline, and pentylenetetrazol) to slices in vitro from PrP knockout (Prnp0/0) and control mice. In all three epilepsy models, we found that longer delays and/or higher concentrations of convulsants were necessary to generate spontaneous epileptiform activity in Prnp0/0 mice. These results together indicate an increased seizure threshold in Prnp0/0 mice, suggesting that loss of PrP function cannot explain a predisposition to seizures initiation in CJD.

KW - prion

KW - epilepsy

KW - hippocampus

KW - pentylenetetrazol

KW - bicuculline

KW - zero-magnesium

U2 - 10.1016/j.neuroscience.2011.01.053

DO - 10.1016/j.neuroscience.2011.01.053

M3 - Article

C2 - 21277354

VL - 179

SP - 56

EP - 61

JO - Neuroscience

JF - Neuroscience

SN - 0306-4522

ER -