Scn1a+/- (Dravet model)
This article discusses the Scn1a [gene] model, a pathological model for Dravet syndrome (epileptic seizures, developmental delay).+/-" features, main research subjects, reviews, and main products are summarized.
SCN1A+/-(Dravet model)
SCN1A+/-IsPathology model of Dravet syndrome (epileptic seizures, developmental delay)This is a disease model mouse that replicates the heterozygous mutation/deletion of the Scn1a gene, which produces the sodium channel alpha subunit type 1 protein "Nav1.1," the main cause of Dravet syndrome. Because it can reproduce symptoms such as epileptic seizures, developmental disorders, and sudden death observed in human Dravet syndrome, it is widely used worldwide as a model for verifying the efficacy of new antiepileptic drugs and gene therapy drugs.
The main subjects of research utilization
| Diseases for research |
How to use a disease model |
| Development of Novel Treatments for Dravet Syndrome |
Administering novel compounds such as antiepileptic drugs and cannabidiol (CBD) to verify reductions in seizure frequency and improvements in survival rates. |
| Gene therapy and nucleic acid drug development |
Proof-of-concept (PoC) study using AAV vectors for gene supplementation and antisense oligonucleotides for protein expression recovery. |
Sudden unexpected death in epilepsy and
Mechanisms of developmental disorders |
Used to elucidate the mechanisms of autonomic nerve disorders such as decreased respiratory function, and accompanying cognitive and behavioral abnormalities. |
This model's primary endpoints are "seizure suppression in vivo" and "extension of survival time."
| How to use |
Details |
| Electroencephalogram (EEG) measurement |
record brain waves, and analyze the frequency and duration of epileptic seizures,
and quantitatively evaluate abnormal waveforms during seizures |
Heat provocation test
(Fever-related seizure assessment) |
When the body temperature of a mouse is artificially raised and febrile seizures are induced,
Measure the threshold body temperature (at what temperature seizures occur) and assess seizure susceptibility. |
Electrophysiological analysis
(Patch clamp method) |
"GABAergic inhibitory interneurons" using brain slices
Measure sodium current density and neural activity to verify functional recovery at the cellular level |
| Pharmacological action test |
Evaluation of ataxia (coordination function) using the rotarod test
Combining open-field tests, social behavior tests, and other methods,
Comprehensive evaluation of the impact on cognitive and behavioral disorders, which are accompanying symptoms of epilepsy |
| Survival rate and survival period tracking |
using Kaplan-Meier survival curves, among others,
Evaluate the survival benefit of drug administration |
SCN1A+/-Review of the Dravet Model
In a mouse model of severe myoclonic epilepsy
Reduced sodium current in GABAergic interneurons
Mutations in voltage-gated sodium channel (NaV1.1) cause severe myoclonic epilepsy of infancy (SMEI/Dravet syndrome). Scn1a−/−In mice, ataxia was observed, they died on day 15 after birth, and Scn1a+/-In mice, spontaneous seizures and sporadic mortality were observed at 21 days of age.
Analysis of hippocampal neurons revealed a significant decrease in sodium current density in GABAergic inhibitory interneurons. In contrast, no decrease was observed in excitatory pyramidal neurons. As a result,Hyperexcitability leading to epilepsy in SMEI may be caused by a decrease in GABAergic neuron function.indicates that there is.
SCN1A+/-(Dravet model) main products
Here, at Google, "Scn1a"+/-(Dravet Model) We searched for "product" and are introducing two products from the top 10 search results where product pages were confirmed (surveyed June 3, 2026).
SCN1A knockout (System Number: 037107)
Knockout mice with exon 1 of the Scn1a gene deleted.Standard models for genetic analysis and preclinical testing of epilepsyAs a widely used model.
| Manufacturer/Distributor |
Analysis items |
Primary Endpoint |
| The Jackson Laboratory |
Frequency of spontaneous epileptic seizures, ataxia (e.g., rotarod test), and survival rate |
Suppression of epileptic seizures, extension of survival time |
SCN1AR613X; Dravet Model #10 (Serial No.: 034129)
ActualFaithfully reproduce specific gene mutations (nonsense mutations) found in Dravet syndrome patientsThis is an "open access" disease model mouse.
| Manufacturer/Distributor |
Analysis items |
Primary Endpoint |
| The Jackson Laboratory |
Scn1a mRNA and Nav1.1 protein expression levels, and epilepsy seizure frequency |
Restoration of protein function by lead-through therapy, etc., suppression of seizures, and improvement of survival rate |
Summary
The Scn1a introduced in this article+/-The model is a mouse that replicates epilepsy and sudden death associated with Dravet syndrome, and is used for evaluating new drugs and gene therapies.In non-clinical studies, selecting a reliable model is a critical point.This site summarizes pathological models used in non-clinical studies, so please refer to the following articles.
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