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iCell GABANeurons SCN1A HZ KO, 01279
Product Overview
SCN1A gene encodes the voltage-gated sodium channel Nav1.1 alpha subunit, which is highly expressed in neurons within the central nervous system. Mutations in SCN1A are associated with idiopathic epilepsy, genetic epilepsy with febrile seizures (GEFS), and are the predominant gene affiliated with Dravet Syndrome. Knockout mouse models of SCN1A show significant effects on GABAergic sodium currents and a reduction in neural inhibition contributing to seizurogenic activity.
Accessible human cellular models that recapitulate SCN1A epilepsy phenotypes are needed for discovery and validation of much-needed therapeutics. iCell GABANeurons SCN1A Heterozygous (HZ) Knockout (KO) provide a useful cellular model for Dravet Syndrome and epilepsy. The SCN1A HZ KO was engineered into induced pluripotent stem cells, followed by differentiation into cortical neurons that exhibit a primarily GABAergic phenotype.
Features of iCell GABANeurons SCN1A HZ KO:
- >95% pure neuronal population of primarily GABAergic neurons
- Display relevant epileptic phenotypes
- Enhanced neural activity phenotypes when co-cultured with iCell GlutaNeurons
- Consistent lot-to-lot GABA phenotypes and cell viability
- Available with isogenic wild-type iCell GABANeurons, 01279
Components:
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Our specialists are here to help you find the best product for your application.
Our regular business hours are 9:00am to 5:00pm Central Time (USA)
Technical Docs
PROTOCOLS
Application Notes
Applying Transfection Technologies to Create Novel Screening Models - iCell Neural Products
Application Protocols
Assessing Neurite Outgrowth: Quantification with High Content Screening
Application Protocols
Immunofluorescent Labeling of iCell GABANeurons
Application Protocols
Plating into 1536-well Cell Culture Plates
Application Protocols
Silencing Gene Expression: siRNA Delivery by Transfection
Application Protocols
Using Liposome-mediated Transfection for Gene Delivery - iCell GABANeurons
USER DOCS
User's Guides
iCell GABANeurons User's Guide
Safety Data Sheet
iCell Cell-Based Products Safety Data Sheet - Asia
Safety Data Sheet
iCell Cell-Based Products Safety Data Sheet
Performance Data
iCell GABANeurons SCN1A KO Neurite Outgrowth is Increased.
Neurons were plated in 96-well format and analyzed at 10 days in vitro for neurite outgrowth and branch points using high-content imaging. A) Representative brightfield images of isogenic control iCell GABANeurons and SCN1A neurons captured on high content imaging. B) Quantification of neurite length over time shows increased outgrowth in SCN1A neurons over isogenic control. Similarly, iCell GABANeurons KCNT1 (P924L), another epilepsy cell line, also show aberrant neurite outgrowth and branching. C) Quantification of neurite branch points over time shows increased neurite branching for SCN1A and KCNT1 over isogenic controls.
Figure 1: iCell GABANeurons SCN1A KO Neurite Outgrowth is Increased
Figure 2: iCell GABANeuron SCN1A KO Exhibit Altered Neural Activity
iCell GABANeuron SCN1A KO Exhibit Altered Neural Activity.
iCell GABANeurons SCN1A KO or isogenic control iCell GABANeurons were cultured and monitored for neural activity via multielectrode array (MEA) following 19 days in culture. A) Representative raster plots show SCN1A KO GABANeurons have altered neural activity compared to isogenic control GABANeurons. Neither SCN1A KO GABANeurons or isogenic control cultures show synchronous network activity as displayed by iCell GlutaNeuron monoculture. B) Quantification of MEA activity shows SCN1A KO GABANeurons have a similar burst rate, increased burst intensity, and decreased burst duration compared to isogenic GABANeuron cultures.
Enhanced Disease Phenotype in iCell GABANeuron SCN1A KO when Co-Cultured with iCell GlutaNeurons.
Isogenic iCell GlutaNeurons were co-cultured with either iCell GABANeurons SCN1A KO or isogenic control iCell GABANeurons at a ratio of 1:3. Neural activity was monitored via multielectrode array (MEA) following 19 days in culture. Raster plots show network burst frequency was increased in SCN1A KO cultures compared to control iCell GABANeurons.
Figure 3: Enhanced Disease Phenotype in iCell GABANeuron SCN1A KO when Co-Cultured with iCell GlutaNeurons
Figure 4: Neural Tri-culture using iCell GABANeurons SCN1A Supports Epilepsy Phenotype
Neural Tri-culture using iCell GABANeurons SCN1A Supports Epilepsy Phenotype.
iCell GlutaNeurons and iCell Astrocytes were cultured with either iCell GABANeurons SCN1A KO or isogenic control iCell GABANeurons at a 2:1:2 ratio. Neural activity was monitored using multielectrode array (MEA). Increased frequency of neural bursting activity was observed in SCN1A KO tri-culture over control GABANeuron cultures.
Product Highlights
Applications:
- Epilepsy disease modeling
- Dravet Syndrome
- Idiopathic epilepsy, genetic epilepsy with febrile seizures
- Seizurogenic compound testing
- Genetic analysis of epilepsy
- Drug target and therapeutic screening