Scn1a-KO Mouse
Product Name
Scn1a-KO Mouse
Product ID
C002015
Strain Name
C57BL/6JCya-Scn1aem1/Cya
Backgroud
C57BL/6JCya
Status
When using this mouse strain in a publication, please cite “Scn1a-KO Mouse (Catalog C002015) were purchased from Cyagen.”
Product Type
Age
Genotype
Sex
Quantity
Gene Name
Scn1a
Gene Alias
Nav1.1, B230332M13
NCBI ID
Chromosome
Chr 2 (Mouse)
MGI ID
Datasheet
Strain Description
The SCN1A gene encodes the α subunit of the voltage-gated sodium channel Nav1.1, which plays a critical role in regulating neuronal excitability and synaptic transmission. Loss-of-function mutations in SCN1A are closely associated with various epilepsy syndromes, most notably Dravet syndrome (DS) [1]. SCN1A mutations primarily affect GABAergic inhibitory interneurons, leading to impaired inhibitory neural circuits, disruption of the excitation/inhibition (E/I) balance, and increased epilepsy susceptibility [2].
The Scn1a-KO mouse model was generated by using gene-editing technology to delete exon 2 of the murine Scn1a gene. This strain is homozygous lethal, with heterozygotes also exhibiting premature mortality. It is suitable for antiepileptic drug screening, validation of SCN1A gene therapy strategies, investigation of epileptogenesis, and research on interventions for neuropsychiatric comorbidities.
Reference
Martins Custodio H, Clayton LM, Bellampalli R, Pagni S, Silvennoinen K, Caswell R; Genomics England Research Consortium; Brunklaus A, Guerrini R, Koeleman BPC, Lemke JR, Møller RS, Scheffer IE, Weckhuysen S, Zara F, Zuberi S, Kuchenbaecker K, Balestrini S, Mills JD, Sisodiya SM. Widespread genomic influences on phenotype in Dravet syndrome, a 'monogenic' condition. Brain. 2023 Sep 1;146(9):3885-3897.
Mattei C, Mao M, Byars S, Syazwan EM, Oliva M, Karle TJ, Richards K, Scheffer IE, Petrou S, Maljevic S. Disrupted inhibitory interneuron development in SCN1A Dravet syndrome revealed by patient-derived subpallial organoids. Epilepsia. 2026 May 5.
Strain Strategy
The murine Scn1a gene, located on chromosome 2, comprises 26 exons. Exon 2 of this gene was targeted for deletion using gene-editing technology.
Figure 1. Diagram of the gene editing strategy for the generation of Scn1a-KO mice.
Application Area
Anti-epileptic drug screening: Used to evaluate the therapeutic efficacy and safety of novel anti-epileptic drugs (e.g., fenfluramine, stiripentol) on thermally induced seizures, spontaneous seizures, and related neurological dysfunctions;
Validation of gene therapy strategies: Used to assess the therapeutic potential of AAV-mediated SCN1A gene replacement therapy, gene editing repair strategies, and transcriptional activation technologies after disease onset;
Epilepsy pathogenesis research: Used to investigate the mechanisms by which SCN1A deficiency leads to reduced excitability of GABAergic interneurons, abnormal inhibitory synaptic transmission, and excitation/inhibition imbalance in neural networks during epilepsy development;
Neuropsychiatric comorbidity research: Used to study the mechanisms and intervention effects of Dravet syndrome (DS)-associated neuropsychiatric comorbidities, including cognitive impairment, abnormal social behavior, anxiety-like behavior, and motor dysfunction;
Disease reversibility research: Used to explore whether restoring Nav1.1 protein expression levels after the appearance of disease phenotypes can reverse epileptic phenotypes, reduce the risk of sudden unexpected death in epilepsy (SUDEP), and improve cognitive and behavioral deficits.
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