scholarly journals Potentiating α 2 subunit containing perisomatic GABA A receptors protects against seizures in a mouse model of Dravet syndrome

2019 ◽  
Vol 597 (16) ◽  
pp. 4293-4307 ◽  
Author(s):  
Toshihiro Nomura ◽  
Nicole A. Hawkins ◽  
Jennifer A. Kearney ◽  
Alfred L. George ◽  
Anis Contractor
Keyword(s):  
Mouse Model ◽  
Dravet Syndrome ◽  
Gaba A

Adolescent behavioral abnormalities in a Scn1a+/− mouse model of Dravet syndrome

2020 ◽  
Vol 103 ◽  
pp. 106842
Author(s):  
Dilara Bahceci ◽  
Lyndsey Leigh Anderson ◽  
Cassandra Veronica Occelli Hanbury Brown ◽  
Cilla Zhou ◽  
Jonathon Carl Arnold
Keyword(s):  
Mouse Model ◽  
Dravet Syndrome ◽  
Behavioral Abnormalities

scholarly journals Metabolomic signature of the Dravet syndrome: A genetic mouse model study

Epilepsia ◽  
2021 ◽  
Author(s):  
Nina Miljanovic ◽  
Roelof Maarten van Dijk ◽  
Verena Buchecker ◽  
Heidrun Potschka
Keyword(s):  
Mouse Model ◽  
Dravet Syndrome ◽  
Genetic Mouse Model ◽  
Model Study

scholarly journals The Heat Sensing Trpv1 Receptor Is Not a Viable Anticonvulsant Drug Target in the Scn1a+/− Mouse Model of Dravet Syndrome

2021 ◽  
Vol 12 ◽  
Author(s):  
Vaishali Satpute Janve ◽  
Lyndsey L. Anderson ◽  
Dilara Bahceci ◽  
Nicole A. Hawkins ◽  
Jennifer A. Kearney ◽  
...  
Keyword(s):  
Mouse Model ◽  
Drug Target ◽  
Genetic Modifier ◽  
Anticonvulsant Drug ◽  
Dravet Syndrome ◽  
Seizure Threshold ◽  
Drug Resistant ◽  
Spontaneous Seizures ◽  
Temperature Thresholds ◽  
Seizure Models

Cannabidiol has been approved for the treatment of drug-resistant childhood epilepsies including Dravet syndrome (DS). Although the mechanism of anticonvulsant action of cannabidiol is unknown, emerging data suggests involvement of the transient receptor potential cation channel subfamily V member 1 (Trpv1). Pharmacological and genetic studies in conventional seizure models suggest Trpv1 is a novel anticonvulsant target. However, whether targeting Trpv1 is anticonvulsant in animal models of drug-resistant epilepsies is not known. Thus, we examined whether Trpv1 affects the epilepsy phenotype of the F1.Scn1a+/− mouse model of DS. We found that cortical Trpv1 mRNA expression was increased in seizure susceptible F1.Scn1a+/− mice with a hybrid genetic background compared to seizure resistant 129.Scn1a+/− mice isogenic on 129S6/SvEvTac background, suggesting Trpv1 could be a genetic modifier. Previous studies show functional loss of Trpv1 is anticonvulsant. However, Trpv1 selective antagonist SB-705498 did not affect hyperthermia-induced seizure threshold, frequency of spontaneous seizures or survival of F1.Scn1a+/− mice. Surprisingly, Trpv1 deletion had both pro- and anti-seizure effects. Trpv1 deletion did not affect hyperthermia-induced seizure temperature thresholds of F1.Scn1a+/−; Trpv1+/− at P14-16 but was proconvulsant at P18 as it reduced seizure temperature thresholds. Conversely, Trpv1 deletion did not alter the frequency of spontaneous seizures but reduced their severity. These results suggest that Trpv1 is a modest genetic modifier of spontaneous seizure severity in the F1.Scn1a+/− model of DS. However, the opposing pro- and anti-seizure effects of Trpv1 deletion and the lack of effects of Trpv1 inhibition suggest that Trpv1 is unlikely a viable anticonvulsant drug target in DS.

scholarly journals Donepezil increases resistance to induced seizures in a mouse model of Dravet syndrome

2019 ◽  
Vol 6 (8) ◽  
pp. 1566-1571 ◽  
Author(s):  
Jennifer C. Wong ◽  
Jacquelyn T. Thelin ◽  
Andrew Escayg
Keyword(s):  
Mouse Model ◽  
Dravet Syndrome

scholarly journals Pharmacokinetics of Phytocannabinoid Acids and Anticonvulsant Effect of Cannabidiolic Acid in a Mouse Model of Dravet Syndrome

2019 ◽  
Vol 82 (11) ◽  
pp. 3047-3055 ◽  
Author(s):  
Lyndsey L. Anderson ◽  
Ivan K. Low ◽  
Samuel D. Banister ◽  
Iain S. McGregor ◽  
Jonathon C. Arnold
Keyword(s):  
Mouse Model ◽  
Dravet Syndrome ◽  
Anticonvulsant Effect ◽  
Cannabidiolic Acid

Characterisation of a Dravet syndrome knock-in mouse model useful for investigating cannabinoid-based treatments

2018 ◽  
Vol 28 ◽  
pp. S41 ◽  
Author(s):  
C. Alonso Gómez ◽  
V. Satta ◽  
J. Fernández-Ruiz ◽  
O. Sagredo
Keyword(s):  
Mouse Model ◽  
Dravet Syndrome

scholarly journals Nav1.1 haploinsufficiency in excitatory neurons ameliorates seizure-associated sudden death in a mouse model of Dravet syndrome

2013 ◽  
Vol 22 (23) ◽  
pp. 4784-4804 ◽  
Author(s):  
Ikuo Ogiwara ◽  
Takuji Iwasato ◽  
Hiroyuki Miyamoto ◽  
Ryohei Iwata ◽  
Tetsushi Yamagata ◽  
...  
Keyword(s):  
Sudden Death ◽  
Mouse Model ◽  
Dravet Syndrome ◽  
Excitatory Neurons

scholarly journals Impaired excitability of somatostatin- and parvalbumin-expressing cortical interneurons in a mouse model of Dravet syndrome

2014 ◽  
Vol 111 (30) ◽  
pp. E3139-E3148 ◽  
Author(s):  
C. Tai ◽  
Y. Abe ◽  
R. E. Westenbroek ◽  
T. Scheuer ◽  
W. A. Catterall
Keyword(s):  
Mouse Model ◽  
Dravet Syndrome ◽  
Cortical Interneurons

scholarly journals Modulating GABA A receptors that contain alpha 2/3 subunits corrects respiratory disturbances in a mouse model of Rett syndrome

2009 ◽  
Vol 23 (S1) ◽  
Author(s):  
John M Bissonnette ◽  
Sharon J Knopp
Keyword(s):  
Mouse Model ◽  
Rett Syndrome ◽  
Gaba A
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