Calpastatin, a calpain specific inhibitor, reduce seizures in a mouse model of temporal lobe epilepsy

Abstract Epilepsy is a chronic condition characterized by unpredictable and recurrent spontaneous seizures. In a previous study, we reported that pharmacological inhibition of calpain prevented epileptogenesis in the rat pilocarpine model. In this study, we demonstrate that transgenic overexpression...

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Main Authors: Philip M. Lam, Mala V. Rao, Ralph A. Nixon, Marco I. González
Format: Article
Language:English
Published: Wiley 2025-06-01
Series:Epilepsia Open
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Online Access:https://doi.org/10.1002/epi4.70030
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author Philip M. Lam
Mala V. Rao
Ralph A. Nixon
Marco I. González
author_facet Philip M. Lam
Mala V. Rao
Ralph A. Nixon
Marco I. González
author_sort Philip M. Lam
collection DOAJ
description Abstract Epilepsy is a chronic condition characterized by unpredictable and recurrent spontaneous seizures. In a previous study, we reported that pharmacological inhibition of calpain prevented epileptogenesis in the rat pilocarpine model. In this study, we demonstrate that transgenic overexpression of calpastatin, the endogenous inhibitor of calpain, reduces calpain activation and lessens seizure burden in the mouse intrahippocampal kainate model. Blockade of calpain activation was evidenced by a reduction in the generation of spectrin breakdown products, a hallmark of calpain activation. CAST overexpression was associated with a significant reduction in seizure burden, further supporting the idea that blocking calpain overactivation prevents epilepsy. Moreover, a reduction in seizure burden was accompanied by a decrease in inflammatory markers but not cell death. Together, these observations corroborate the role of calpain overactivation in epileptogenesis and provide further support for the use of calpain inhibitors as a viable strategy to prevent epilepsy. Plain Language Summary The mechanisms by which brain alterations lead to spontaneous seizures are not well understood. Acquired epilepsy often follows brain trauma. After a brain injury, the activation of the protease calpain has been associated with the development of spontaneous seizures. Our observations indicate that transgenic overexpression of calpastatin, an endogenous inhibitor of calpain, impacts epileptogenesis and reduces seizure burden. This suggests that inhibiting calpain could be a viable strategy to prevent epilepsy.
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spelling doaj-art-715b19884cd24eb18b3add59ce46d02b2025-08-20T03:21:17ZengWileyEpilepsia Open2470-92392025-06-0110395796410.1002/epi4.70030Calpastatin, a calpain specific inhibitor, reduce seizures in a mouse model of temporal lobe epilepsyPhilip M. Lam0Mala V. Rao1Ralph A. Nixon2Marco I. González3Department of Pediatrics, Division of Neurology University of Colorado School of Medicine Aurora Colorado USACenter for Dementia Research and Department of Psychiatry and Cell Biology New York University Langone Medical Center New York New York USACenter for Dementia Research and Department of Psychiatry and Cell Biology New York University Langone Medical Center New York New York USADepartment of Pediatrics, Division of Neurology University of Colorado School of Medicine Aurora Colorado USAAbstract Epilepsy is a chronic condition characterized by unpredictable and recurrent spontaneous seizures. In a previous study, we reported that pharmacological inhibition of calpain prevented epileptogenesis in the rat pilocarpine model. In this study, we demonstrate that transgenic overexpression of calpastatin, the endogenous inhibitor of calpain, reduces calpain activation and lessens seizure burden in the mouse intrahippocampal kainate model. Blockade of calpain activation was evidenced by a reduction in the generation of spectrin breakdown products, a hallmark of calpain activation. CAST overexpression was associated with a significant reduction in seizure burden, further supporting the idea that blocking calpain overactivation prevents epilepsy. Moreover, a reduction in seizure burden was accompanied by a decrease in inflammatory markers but not cell death. Together, these observations corroborate the role of calpain overactivation in epileptogenesis and provide further support for the use of calpain inhibitors as a viable strategy to prevent epilepsy. Plain Language Summary The mechanisms by which brain alterations lead to spontaneous seizures are not well understood. Acquired epilepsy often follows brain trauma. After a brain injury, the activation of the protease calpain has been associated with the development of spontaneous seizures. Our observations indicate that transgenic overexpression of calpastatin, an endogenous inhibitor of calpain, impacts epileptogenesis and reduces seizure burden. This suggests that inhibiting calpain could be a viable strategy to prevent epilepsy.https://doi.org/10.1002/epi4.70030calpainepileptogenesisIHKAproteolysisseizures
spellingShingle Philip M. Lam
Mala V. Rao
Ralph A. Nixon
Marco I. González
Calpastatin, a calpain specific inhibitor, reduce seizures in a mouse model of temporal lobe epilepsy
Epilepsia Open
calpain
epileptogenesis
IHKA
proteolysis
seizures
title Calpastatin, a calpain specific inhibitor, reduce seizures in a mouse model of temporal lobe epilepsy
title_full Calpastatin, a calpain specific inhibitor, reduce seizures in a mouse model of temporal lobe epilepsy
title_fullStr Calpastatin, a calpain specific inhibitor, reduce seizures in a mouse model of temporal lobe epilepsy
title_full_unstemmed Calpastatin, a calpain specific inhibitor, reduce seizures in a mouse model of temporal lobe epilepsy
title_short Calpastatin, a calpain specific inhibitor, reduce seizures in a mouse model of temporal lobe epilepsy
title_sort calpastatin a calpain specific inhibitor reduce seizures in a mouse model of temporal lobe epilepsy
topic calpain
epileptogenesis
IHKA
proteolysis
seizures
url https://doi.org/10.1002/epi4.70030
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AT ralphanixon calpastatinacalpainspecificinhibitorreduceseizuresinamousemodeloftemporallobeepilepsy
AT marcoigonzalez calpastatinacalpainspecificinhibitorreduceseizuresinamousemodeloftemporallobeepilepsy