{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,6]],"date-time":"2026-11-06T01:07:44Z","timestamp":1793927264867,"version":"4.8.0"},"reference-count":136,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2025,7,23]],"date-time":"2025-07-23T00:00:00Z","timestamp":1753228800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Neurosci."],"abstract":"<jats:p>\n                    Dravet Syndrome (DS), a rare genetic encephalopathy characterized by severe drug-resistant epilepsy and progressive neurodevelopmental regression in infancy, is caused by\n                    <jats:italic>de novo<\/jats:italic>\n                    mutations in the\n                    <jats:italic>SCN1A<\/jats:italic>\n                    gene on chromosome 2q24 in over 80% of cases. This review synthesizes current insights into its molecular pathogenesis, precision diagnostics, and therapeutic innovations:\n                    <jats:italic>SCN1A<\/jats:italic>\n                    mutations disrupt Nav1.1 sodium channel expression and membrane trafficking in GABAergic interneurons through transcriptional dysregulation, pre-mRNA splicing defects, and gating dysfunction, thereby impairing inhibitory synaptic transmission and disrupting brainwide excitatory-inhibitory balance. Notably, polygenic interactions (e.g.,\n                    <jats:italic>DEPDC5<\/jats:italic>\n                    ,\n                    <jats:italic>CHD2<\/jats:italic>\n                    variants), astrocytic calcium signaling aberrations, and mitochondrial metabolic deficits synergistically exacerbate network hyperexcitability. Diagnostic advancements include a stratified framework integrating early febrile seizure phenotypes, comprehensive\n                    <jats:italic>SCN1A<\/jats:italic>\n                    sequencing (including deep intronic variants), and multimodal assessments (e.g.,\n                    <jats:italic>\u03b3<\/jats:italic>\n                    -band EEG power analysis and hippocampal volumetry), which significantly accelerate clinical diagnosis and reduce misdiagnosis. Therapeutic strategies are evolving from empirical seizure control to mechanism-targeted interventions: antisense oligonucleotides (ASOs) restore\n                    <jats:italic>SCN1A<\/jats:italic>\n                    transcript integrity by blocking pathogenic exon inclusion; adeno-associated virus (AAV9)-mediated activation of GABAergic neuron-specific\n                    <jats:italic>SCN1A<\/jats:italic>\n                    promoters and CRISPR\/dCas9-driven endogenous Nav1.1 upregulation have both been shown to improve inhibitory synaptic function and elevate seizure thresholds in preclinical models. Additionally, novel molecules such as the Nav1.1-selective agonist Hm1a and 5HT\n                    <jats:sub>2<\/jats:sub>\n                    BR receptor modulators offer new avenues by remodeling neuronal electrophysiology and neurotransmitter homeostasis. By dissecting the multi-dimensional molecular networks underlying DS and highlighting interdisciplinary integration of diagnostic-therapeutic technologies, this review provides a theoretical foundation for developing\n                    <jats:italic>SCN1A<\/jats:italic>\n                    -centric precision medicine, advocating a shift from symptomatic management to mechanism-driven interventions in clinical practice.\n                  <\/jats:p>","DOI":"10.3389\/fnins.2025.1634718","type":"journal-article","created":{"date-parts":[[2025,7,23]],"date-time":"2025-07-23T13:50:51Z","timestamp":1753278651000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":17,"title":["Dravet syndrome: novel insights into SCN1A-mediated epileptic neurodevelopmental disorders within the molecular diagnostic-therapeutic framework"],"prefix":"10.3389","volume":"19","author":[{"given":"Guirui","family":"Zhang","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shupeng","family":"Huang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mingzhen","family":"Wei","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yongmo","family":"Wu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zhengyi","family":"Xie","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jin","family":"Wang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2025,7,23]]},"reference":[{"key":"ref2","doi-asserted-by":"publisher","first-page":"105209","DOI":"10.1016\/j.nbd.2020.105209","article-title":"Developmental alterations in firing properties of hippocampal CA1 inhibitory and excitatory neurons in a mouse model of Dravet syndrome","volume":"148","author":"Almog","year":"2021","journal-title":"Neurobiol. 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