Double Trouble: Impairment of Two Interneuron Types in a Dravet Mouse Model: Impairment of Multiple Interneurons in Dravet Mice

JA Kearney�- Epilepsy Currents, 2015 - journals.sagepub.com
Impairment of Multiple Interneurons in Dravet Mice where they exert powerful feed-forward
inhibition. Martinotti cells target the apical dendrites of layer V tufted pyramidal neurons and�…

[HTML][HTML] New complex physiological findings evolve hypothesized mechanisms of Dravet syndrome

MKA Howard�- Neural Regeneration Research, 2024 - journals.lww.com
Developmental and epileptic encephalopathies (DEEs) are neurological disorders generally
involving medically intractable seizures and a diverse array of comorbid neuropsychiatric�…

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�…

[HTML][HTML] GABA tonic currents and glial cells are altered during epileptogenesis in a mouse model of Dravet syndrome

RC Goisis, A Chiavegato, M Gomez-Gonzalo…�- Frontiers in cellular�…, 2022 - frontiersin.org
Dravet Syndrome (DS) is a rare autosomic encephalopathy with epilepsy linked to Nav1. 1
channel mutations and defective GABAergic signaling. Effective therapies for this syndrome�…

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�…

Potentiating NaV1.1 in Dravet syndrome patient iPSC-derived GABAergic neurons increases neuronal firing frequency and decreases network synchrony

MR Kelley, LB Chipman, S Asano, M Knott, ST Howard…�- bioRxiv, 2023 - biorxiv.org
Dravet syndrome is a developmental and epileptic encephalopathy characterized by
seizures, behavioral abnormalities, developmental deficits, and elevated risk of sudden�…

[HTML][HTML] Dravet Variant SCN1AA1783V Impairs Interneuron Firing Predominantly by Altered Channel Activation

N Layer, L Sonnenberg, E Pardo Gonz�lez…�- Frontiers in cellular�…, 2021 - frontiersin.org
Dravet syndrome (DS) is a developmental epileptic encephalopathy mainly caused by
functional NaV1. 1 haploinsufficiency in inhibitory interneurons. Recently, a new conditional�…

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�…

Corticohippocampal circuit dysfunction in a mouse model of Dravet syndrome

J Mattis, A Somarowthu, KM Goff, J Yom, NP Sotuyo…�- bioRxiv, 2021 - biorxiv.org
Dravet syndrome (DS) is a neurodevelopmental disorder defined by treatment-resistant
epilepsy, autism spectrum disorder, and sudden death, due to pathogenic variants in�…

Unspooling the Thread: VIP Interneurons Linked With Autism Spectrum Disorder Behaviors but Not Seizures in Dravet Syndrome

R Miralles, MK Patel�- Epilepsy Currents, 2024 - journals.sagepub.com
VIP Interneuron Impairment Promotes In Vivo Circuit Dysfunction and Autism-Related
Behaviors in Dravet Syndrome Goff KM, Liebergall SR, Jiang E, Somarowthu A, Goldberg�…