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

C Tai, Y Abe, RE Westenbroek…�- Proceedings of the�…, 2014 - National Acad Sciences
Haploinsufficiency of the voltage-gated sodium channel NaV1. 1 causes Dravet syndrome,
an intractable developmental epilepsy syndrome with seizure onset in the first year of life�…

A transient developmental window of fast-spiking interneuron dysfunction in a mouse model of Dravet syndrome

M Favero, NP Sotuyo, E Lopez, JA Kearney…�- Journal of�…, 2018 - Soc Neuroscience
Dravet syndrome is a severe, childhood-onset epilepsy largely due to heterozygous loss-of-
function mutation of the gene SCN1A, which encodes the type 1 neuronal voltage-gated�…

Specific deletion of NaV1.1 sodium channels in inhibitory interneurons causes seizures and premature death in a mouse model of Dravet syndrome

CS Cheah, FH Yu, RE Westenbroek…�- Proceedings of the�…, 2012 - National Acad Sciences
Heterozygous loss-of-function mutations in the brain sodium channel NaV1. 1 cause Dravet
syndrome (DS), a pharmacoresistant infantile-onset epilepsy syndrome with comorbidities of�…

Dissecting the phenotypes of Dravet syndrome by gene deletion

M Rubinstein, S Han, C Tai, RE Westenbroek…�- Brain, 2015 - academic.oup.com
Neurological and psychiatric syndromes often have multiple disease traits, yet it is unknown
how such multi-faceted deficits arise from single mutations. Haploinsufficiency of the voltage�…

[HTML][HTML] Developmentally regulated impairment of parvalbumin interneuron synaptic transmission in an experimental model of Dravet syndrome

K Kaneko, CB Currin, KM Goff, ER Wengert…�- Cell reports, 2022 - cell.com
Dravet syndrome is a neurodevelopmental disorder characterized by epilepsy, intellectual
disability, and sudden death due to pathogenic variants in SCN1A with loss of function of the�…

Dravet syndrome: a sodium channel interneuronopathy

WA Catterall�- Ion Channels in Health and Disease, 2016 - Elsevier
Voltage-gated sodium channels initiate action potentials (APs) in brain neurons, and sodium
channel blockers are used to treat epilepsy. Heterozygous loss-of-function mutations in Na V�…

Genetic background modulates impaired excitability of inhibitory neurons in a mouse model of Dravet syndrome

M Rubinstein, RE Westenbroek, HY Frank…�- Neurobiology of�…, 2015 - Elsevier
Dominant loss-of-function mutations in voltage-gated sodium channel Na V 1.1 cause
Dravet Syndrome, an intractable childhood-onset epilepsy. Na V 1.1+/− Dravet Syndrome�…

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

I Ogiwara, T Iwasato, H Miyamoto…�- Human molecular�…, 2013 - academic.oup.com
Dravet syndrome is a severe epileptic encephalopathy mainly caused by heterozygous
mutations in the SCN1A gene encoding a voltage-gated sodium channel Nav1. 1. We�…

Insights into pathophysiology and therapy from a mouse model of Dravet syndrome

JC Oakley, F Kalume, WA Catterall�- Epilepsia, 2011 - Wiley Online Library
Mutations in voltage‐gated sodium channels are associated with epilepsy syndromes with a
wide range of severity. Complete loss of function in the Nav1. 1 channel encoded by the�…

[HTML][HTML] Developmental alterations in firing properties of hippocampal CA1 inhibitory and excitatory neurons in a mouse model of Dravet syndrome

Y Almog, S Fadila, M Brusel, A Mavashov…�- Neurobiology of�…, 2021 - Elsevier
Dravet syndrome (Dravet) is a rare, severe childhood-onset epilepsy, caused by
heterozygous de novo mutations in the SCN1A gene, encoding for the alpha subunit of the�…