Heat induced temperature dysregulation and seizures in Dravet Syndrome/GEFS+ Gabrg2+/Q390X mice

Epilepsy Research
Timothy A WarnerJing-Qiong Kang

Abstract

It has been established that febrile seizures and its extended syndromes like generalized epilepsy with febrile seizures (FS) plus (GEFS+) and Dravet syndrome have been associated with mutations especially in SCN1A and GABRG2 genes. In patients, the onset of FS is likely due to the combined effect of temperature and inflammation in genetically vulnerable individuals because fever is often associated with infection. Much effort has been spent to understand the mechanisms underlying fever induction of seizures. In addition to the role of cytokines in FS, previous studies in Scn1a+/- knockout mice, a model of Dravet syndrome, indicated that temperature elevation alone could result in seizure generation, and the effect of elevated temperature inducing seizures was age-dependent. Here, we report the thermal effect in a different mouse model of Dravet syndrome, the Gabrg2+/Q390X knockin mouse. We demonstrated age-dependent dysregulated temperature control and that temperature elevation produced myoclonic jerks, generalized tonic clonic seizures (GTCSs) and heightened anxiety-like symptoms in Gabrg2+/Q390X mice. The study indicated that regardless of other inflammatory factors, brief heat alone increased brain excitability and induced...Continue Reading

References

Aug 1, 1992·International Journal of Developmental Neuroscience : the Official Journal of the International Society for Developmental Neuroscience·G el HamdiA Nehlig
Feb 8, 2002·American Journal of Physiology. Regulatory, Integrative and Comparative Physiology·Silvia GattiCharles A Dinarello
Jul 16, 2002·Archives of Neurology·Colette KananuraOrtrud K Steinlein
Dec 13, 2002·Brain : a Journal of Neurology·Carla MariniSamuel F Berkovic
Oct 24, 2003·Molecular and Cellular Neurosciences·Claude SchweizerBernhard Lüscher
Jul 2, 2004·The Journal of Neuroscience : the Official Journal of the Society for Neuroscience·Cheryl A KellerBernhard Lüscher
Dec 29, 2004·Annals of Neurology·Celine DubéTallie Z Baram
Jan 22, 2005·The Journal of Neuroscience : the Official Journal of the Society for Neuroscience·Melissa J AlldredBernhard Lüscher
Mar 25, 2005·The Journal of Neuroscience : the Official Journal of the Society for Neuroscience·David StellwagenRobert C Malenka
Aug 3, 2005·The Journal of Clinical Investigation·Miriam H Meisler, Jennifer A Kearney
Mar 3, 2006·The Journal of Neuroscience : the Official Journal of the Society for Neuroscience·Jing-Qiong KangRobert L Macdonald
Aug 23, 2006·Neurology·D AudenaertP De Jonghe
Nov 23, 2006·Journal of Receptor and Signal Transduction Research·Hanns Möhler
Oct 12, 2007·The Journal of Neuroscience : the Official Journal of the Society for Neuroscience·Raffaella RusconiMassimo Mantegazza
Jun 4, 2008·Pediatrics·UNKNOWN Steering Committee on Quality Improvement and Management, Subcommittee on Febrile Seizures American Academy of Pediatrics
Jan 20, 2009·Neurobiology of Disease·Wendy K ChungDane M Chetkovich
Feb 24, 2009·Proceedings of the National Academy of Sciences of the United States of America·John C OakleyWilliam A Catterall
Sep 11, 2009·Annals of Neurology·Evan A ThomasSteven Petrou
Jun 4, 2010·The Journal of Neuroscience : the Official Journal of the Society for Neuroscience·Céline M DubéTallie Z Baram
Feb 15, 2013·Neurology·Christopher A ReidSteven Petrou
May 26, 2015·Nature Neuroscience·Jing-Qiong KangRobert L Macdonald
Oct 2, 2015·Acta Neuropathologica·Annamaria VezzaniWolfgang Löscher

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Citations

Sep 4, 2020·Journal of Neurochemistry·Thomas J TurnerGabriele Lignani
May 30, 2020·Brain Communications·Shimian QuRobert L Macdonald
Dec 11, 2019·Epilepsia Open·Sanjay M SisodiyaAideen Foley
Dec 18, 2020·Frontiers in Cell and Developmental Biology·Alexandra Colón-RodríguezMegan Y Dennis
Feb 16, 2020·Neuroscience·Merrick S Fallah, James H Eubanks
May 1, 2021·Journal of Clinical Medicine·Justyna PaprockaTomasz Mazurczak

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