Efficacy of stiripentol in hyperthermia-induced seizures in a mouse model of Dravet syndrome

Epilepsia
Dezhi CaoYushi Inoue

Abstract

We previously reported a mutant mouse carrying a severe myoclonic epilepsy in infancy (SMEI) mutation in Scn1a. In this study, we examined the susceptibility to hyperthermia-induced seizures of heterozygous Scn1a mutant mice (Scn1a(RX/+)) and wild-type (Scn1a(+/+) ) mice. Then we assessed the efficacy of stiripentol (STP) monotherapy versus STP and clobazam (CLB) combination therapy to prevent hyperthermia-induced seizures in Scn1a(RX/+) mice. The seizure-inducing body temperatures in Scn1a(RX/+) mice and age-matched Scn1a(+/+) mice were compared in three age groups (1 month, 3-5 months, > 6 months). Then STP, CLB, or STP + CLB was administered intraperitoneally to Scn1a(RX/+) mice of two age groups (p1M, aged 1 month; p5M, aged 5-10 months). The efficacy of medications was assessed by comparing the seizure-inducing body temperature and the duration of seizures. The seizure-inducing body temperature was significantly lower in Scn1a(RX/+) than in Scn1a(+/+) mice for all age groups (p < 0.01). The seizure-inducing body temperature was significantly elevated after administration of STP in p1M (p < 0.05) but not in p5M (p > 0.05), and it was significantly elevated after administration of CLB in both age groups (p < 0.05). The seizu...Continue Reading

References

Jan 1, 1979·British Journal of Clinical Pharmacology·M VolzF Cavagna
Jun 1, 1991·The Japanese Journal of Psychiatry and Neurology·Y OhtsukaS Ohtahara
Nov 1, 1984·Clinical Pharmacology and Therapeutics·R H LevyT A Moreland
Sep 4, 2001·Nihon yakurigaku zasshi. Folia pharmacologica Japonica·H Nakajima
May 20, 2003·Human Mutation·Lieve ClaesPeter De Jonghe
Dec 4, 2003·British Journal of Clinical Pharmacology·N CazaliG Pons
Nov 6, 2004·Journal of Child Neurology·Berten CeulemansLieven Lagae
May 25, 2005·Seizure : the Journal of the British Epilepsy Association·Robert P KustraUNKNOWN TARGET Study Group
Jul 19, 2005·Expert Opinion on Investigational Drugs·Catherine Chiron
Jan 18, 2006·Drug Metabolism and Disposition : the Biological Fate of Chemicals·Carole GiraudAgnès Tran
Jun 1, 2007·The Journal of Neuroscience : the Official Journal of the Society for Neuroscience·Ikuo OgiwaraKazuhiro Yamakawa
Feb 24, 2009·Proceedings of the National Academy of Sciences of the United States of America·John C OakleyWilliam A Catterall
Jun 26, 2009·Epilepsia·Yushi InoueShunya Ikeda

❮ Previous
Next ❯

Citations

Sep 5, 2013·Nature Communications·Scott C BarabanGabriela A Hortopan
Apr 22, 2014·Brain : a Journal of Neurology·Christopher A ReidSteven Petrou
Mar 5, 2016·Expert Opinion on Pharmacotherapy·Catherine Chiron
Nov 2, 2016·Frontiers in Pharmacology·Jennifer C WongAndrew Escayg
Jan 13, 2018·The Journal of Clinical Investigation·YuJaung KimGeorge B Richerson
Mar 23, 2019·Expert Review of Neurotherapeutics·Catherine Chiron
Dec 22, 2017·Pharmacological Reviews·Julia OyrerChristopher A Reid
Aug 5, 2018·Proceedings of the National Academy of Sciences of the United States of America·Kay L RichardsSteven Petrou
Sep 7, 2018·Molecular Brain·Jing LiuBaomin Li
Sep 4, 2018·Communications Biology·Ikuo OgiwaraKazuhiro Yamakawa
May 12, 2017·Annals of Clinical and Translational Neurology·Nicole A HawkinsJennifer A Kearney
Dec 25, 2019·European Journal of Paediatric Neurology : EJPN : Official Journal of the European Paediatric Neurology Society·Alfredo Gonzalez-Sulser
Mar 27, 2021·Physiological Reviews·Massimo MantegazzaWilliam A Catterall
Aug 3, 2021·Frontiers in Pharmacology·Shih-Yin HoHorng-Huei Liou

❮ Previous
Next ❯

Related Concepts

Trending Feeds

COVID-19

Coronaviruses encompass a large family of viruses that cause the common cold as well as more serious diseases, such as the ongoing outbreak of coronavirus disease 2019 (COVID-19; formally known as 2019-nCoV). Coronaviruses can spread from animals to humans; symptoms include fever, cough, shortness of breath, and breathing difficulties; in more severe cases, infection can lead to death. This feed covers recent research on COVID-19.

Blastomycosis

Blastomycosis fungal infections spread through inhaling Blastomyces dermatitidis spores. Discover the latest research on blastomycosis fungal infections here.

Nuclear Pore Complex in ALS/FTD

Alterations in nucleocytoplasmic transport, controlled by the nuclear pore complex, may be involved in the pathomechanism underlying multiple neurodegenerative diseases including Amyotrophic Lateral Sclerosis and Frontotemporal Dementia. Here is the latest research on the nuclear pore complex in ALS and FTD.

Applications of Molecular Barcoding

The concept of molecular barcoding is that each original DNA or RNA molecule is attached to a unique sequence barcode. Sequence reads having different barcodes represent different original molecules, while sequence reads having the same barcode are results of PCR duplication from one original molecule. Discover the latest research on molecular barcoding here.

Chronic Fatigue Syndrome

Chronic fatigue syndrome is a disease characterized by unexplained disabling fatigue; the pathology of which is incompletely understood. Discover the latest research on chronic fatigue syndrome here.

Evolution of Pluripotency

Pluripotency refers to the ability of a cell to develop into three primary germ cell layers of the embryo. This feed focuses on the mechanisms that underlie the evolution of pluripotency. Here is the latest research.

Position Effect Variegation

Position Effect Variagation occurs when a gene is inactivated due to its positioning near heterochromatic regions within a chromosome. Discover the latest research on Position Effect Variagation here.

STING Receptor Agonists

Stimulator of IFN genes (STING) are a group of transmembrane proteins that are involved in the induction of type I interferon that is important in the innate immune response. The stimulation of STING has been an active area of research in the treatment of cancer and infectious diseases. Here is the latest research on STING receptor agonists.

Microbicide

Microbicides are products that can be applied to vaginal or rectal mucosal surfaces with the goal of preventing, or at least significantly reducing, the transmission of sexually transmitted infections. Here is the latest research on microbicides.