Scn1b deletion in adult mice results in seizures and SUDEP

Abstract Pathogenic loss‐of‐function variants in SCN1B are linked to Dravet syndrome (DS). Previous work suggested that neuronal pathfinding defects underlie epileptogenesis and SUDEP in the Scn1b null mouse model of DS. We tested this hypothesis by inducing Scn1b deletion in adult mice that had dev...

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Main Authors:	Heather A. O'Malley, Jacob M. Hull, Brittany C. Clawson, Chunling Chen, Gic Owens‐Fiestan, Margaret B. Jameson, Sara J. Aton, Jack M. Parent, Lori L. Isom
Format:	Article
Language:	English
Published:	Wiley 2019-06-01
Series:	Annals of Clinical and Translational Neurology
Online Access:	https://doi.org/10.1002/acn3.785

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spelling	doaj-f5bde058212248008bc631fb1ce63f5d2021-05-02T08:50:47ZengWileyAnnals of Clinical and Translational Neurology2328-95032019-06-01661121112610.1002/acn3.785Scn1b deletion in adult mice results in seizures and SUDEPHeather A. O'Malley0Jacob M. Hull1Brittany C. Clawson2Chunling Chen3Gic Owens‐Fiestan4Margaret B. Jameson5Sara J. Aton6Jack M. Parent7Lori L. Isom8Department of Pharmacology University of Michigan Ann Arbor Michigan 48109Neuroscience Graduate Program University of Michigan Ann Arbor Michigan 48109Department of Molecular, Cellular, and Developmental Biology University of Michigan Ann Arbor Michigan 48109Department of Pharmacology University of Michigan Ann Arbor Michigan 48109Department of Neurology University of Michigan Ann Arbor Michigan 48109Department of Neurology University of Michigan Ann Arbor Michigan 48109Neuroscience Graduate Program University of Michigan Ann Arbor Michigan 48109Neuroscience Graduate Program University of Michigan Ann Arbor Michigan 48109Department of Pharmacology University of Michigan Ann Arbor Michigan 48109Abstract Pathogenic loss‐of‐function variants in SCN1B are linked to Dravet syndrome (DS). Previous work suggested that neuronal pathfinding defects underlie epileptogenesis and SUDEP in the Scn1b null mouse model of DS. We tested this hypothesis by inducing Scn1b deletion in adult mice that had developed normally. Epilepsy and SUDEP, which occur by postnatal day 21 in Scn1b null animals, were observed within 20 days of induced Scn1b deletion in adult mice, suggesting that epileptogenesis in SCN1B‐DS does not result from defective brain development. Thus, the developmental brain defects observed previously in Scn1b null mice may model other co‐morbidities of DS.https://doi.org/10.1002/acn3.785
collection	DOAJ
language	English
format	Article
sources	DOAJ
author	Heather A. O'Malley Jacob M. Hull Brittany C. Clawson Chunling Chen Gic Owens‐Fiestan Margaret B. Jameson Sara J. Aton Jack M. Parent Lori L. Isom
spellingShingle	Heather A. O'Malley Jacob M. Hull Brittany C. Clawson Chunling Chen Gic Owens‐Fiestan Margaret B. Jameson Sara J. Aton Jack M. Parent Lori L. Isom Scn1b deletion in adult mice results in seizures and SUDEP Annals of Clinical and Translational Neurology
author_facet	Heather A. O'Malley Jacob M. Hull Brittany C. Clawson Chunling Chen Gic Owens‐Fiestan Margaret B. Jameson Sara J. Aton Jack M. Parent Lori L. Isom
author_sort	Heather A. O'Malley
title	Scn1b deletion in adult mice results in seizures and SUDEP
title_short	Scn1b deletion in adult mice results in seizures and SUDEP
title_full	Scn1b deletion in adult mice results in seizures and SUDEP
title_fullStr	Scn1b deletion in adult mice results in seizures and SUDEP
title_full_unstemmed	Scn1b deletion in adult mice results in seizures and SUDEP
title_sort	scn1b deletion in adult mice results in seizures and sudep
publisher	Wiley
series	Annals of Clinical and Translational Neurology
issn	2328-9503
publishDate	2019-06-01
description	Abstract Pathogenic loss‐of‐function variants in SCN1B are linked to Dravet syndrome (DS). Previous work suggested that neuronal pathfinding defects underlie epileptogenesis and SUDEP in the Scn1b null mouse model of DS. We tested this hypothesis by inducing Scn1b deletion in adult mice that had developed normally. Epilepsy and SUDEP, which occur by postnatal day 21 in Scn1b null animals, were observed within 20 days of induced Scn1b deletion in adult mice, suggesting that epileptogenesis in SCN1B‐DS does not result from defective brain development. Thus, the developmental brain defects observed previously in Scn1b null mice may model other co‐morbidities of DS.
url	https://doi.org/10.1002/acn3.785
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Scn1b deletion in adult mice results in seizures and SUDEP

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