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iCell GABANeurons KCNT1 (P924L), 01434

Human iPSC-derived GABA neurons from a Caucasian female (age <18) with an epilepsy disease phenotype (genotype: KCNT1 (P924L), differentiated from fibroblast-derived iPSCs.

Advance epilepsy research with iCell® GABANeurons KCNT1 (P924L), human induced pluripotent stem cell (iPSC)-derived GABAergic neurons.

  • High purity iCell GABANeurons KCNT1 offer >95% pure neuronal populations, ensuring reliable research.
  • Disease relevance The homozygous P924L mutation reproduces the epilepsy-associated gain-of-function increase in KNa1.1 potassium current.
  • Reliable performance Consistent lot-to-lot GABA phenotypes and cell viability drive reproducible research outcomes.
  • Isogenic control Pair with isogenic iCell GABANeurons for confident interpretation of mutation effects.
  • Co-culture compatible iCell GABANeurons KCNT1 support co-culture with iCell GlutaNeurons for a more physiologically relevant model.
Catalog # P924L-01434
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  • 1 vial Cryopreserved iCell GABANeurons KCNT1 (P924L), 01434
  • 100 ml Neural Base Medium 1
  • 2 ml Neural Supplement A
  • User’s Guide

genetic status

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Catalog #:

Epilepsy differentiated from human iPS cells, frozen

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$2,545.00

Cells Only

  • 1 vial Cryopreserved iCell GABANeurons KCNT1 (P924L), 01434
  • User’s Guide

genetic status

Quantity (Cells Per Vials)

Catalog #

Catalog #:

Epilepsy differentiated from human iPS cells, frozen

From
$2,445.00

Product Overview

iCell® GABANeurons are a highly pure population of human neurons derived from induced pluripotent stem (iPS) cells using CDI’s proprietary differentiation and purification protocols. iCell GABANeurons are a mixture of post-mitotic neural subtypes, comprised primarily of GABAergic neurons, with typical physiological characteristics and responses. These cells provide a reliable source of human neurons suitable for use in targeted drug discovery, toxicity testing, and other life science research.

When handled and maintained as recommended, iCell GABANeurons quickly assume a typical neuronal morphology with branching neurites. In addition, these cells display a stable adherent single-cell morphology and remain viable for an extended culture period (≥14 days), making them amenable to a variety of electrophysiology, neurotoxicity, and neurotransmission assays.

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Need Help With This Product?

Our specialists are here to help you find the best product for your application.

Call Us

(877) 320-6688

 

OR

Our regular business hours are 9:00am to 5:00pm Central Time (USA)

Performance Data

KCNT1 (P924L) iCell GABANeurons Viability is Comparable Isogenic Control.

Isogenic control (iCell GABANeurons, 01434) and KCNT1 (P924L) iCell GABANeurons were cultured at equivalent cell densities for 7 days and analyzed for cell viability. A) Representative images of KCNT1 (P924L) GABANeurons and isogenic control GABANeurons stained with Calcein AM (green) and DAPI (blue). B) Quantification of Calcein AM showed similar numbers of viable KCNT1(P924L) iCell GABANeurons compared to isogenic control.

Figure 1: KCNT1 (P924L) iCell GABANeurons Viability is Comparable Isogenic Control

Figure 2: Increased Neurite Growth and Branching for KCNT1 (P924L) iCell GABANeurons

Increased Neurite Growth and Branching for KCNT1 (P924L) iCell GABANeurons.

Isogenic control (iCell GABANeurons, 01434) and iCell GABANeurons KCNT1 (P924L) were cultured at equivalent cell densities and analyzed for neurite outgrowth by high content imaging. A) Representative brightfield images of KCNT1 (P924L) iCell GABANeurons and isogenic control GABANeurons at 7 days in culture. B) KCNT1(P924L) iCell GABANeurons display increased mean neurite outgrowth, branch number and maximum process length compared to isogenic control.

iCell GABANeurons KCNT1 (P924L) Display Expected Action Potential Waveform Pathophysiology.

A) Representative whole-cell current-clamp patch recordings of evoked action potentials (AP) at multiple currents (inset) for iCell GABANeurons KCNT1 (P924L) and isogenic control (iCell GABANeurons, 01434). Whole-cell current-clamp recordings show that (B) AP spike-width is decreased, (C) fast after-hyperpolarization (AHP) is increased, and (D) total evoked APs via current steps (upper graph insets) are increased in KCNT1 (P924L) iCell GABANeurons compared to isogenic GABANeurons. These pathological characteristics align with the ‘gain-of-function’ phenotype observed in many KCNT1 mutations, resulting in a hyper-excitable cell-state.

Figure 3: iCell GABANeurons KCNT1 (P924L) Display Expected Action Potential Waveform Pathophysiology

Figure 4: Network Activity is Increased in iCell GABANeurons KCNT1 (P924L)

Network Activity is Increased in iCell GABANeurons KCNT1 (P924L).

KCNT1 (P924L) GABANeurons and isogenic control (iCell GABANeurons) were cultured and monitored for network activity via MEA. A) Representative raster plots of KCNT1 (P924L) and isogenic control neurons. Analysis of firing rate and intensity show that KCNT1 (P924L) GABANeurons have increased synchrony as indicated by burst intensity (B) and an increased mean firing rate (C) compared to isogenic GABANeurons. These data suggest KCNT1 (P924L) neurons are more “epileptic” than isogenic neurons.

Quinidine Attenuates Activity in KCNT1 (P924L) iCell GABANeurons.

iCell GABANeurons KCNT1 (P924L) or isogenic control iCell GABANeurons were cultured for 15 days prior to treatment with Quinidine (50, 100, or 200 µM), a KCNT1 channel blocker. Following 24 hours, neural activity was recorded by multielectrode array (MEA) and analyzed for mean firing rate (Hz) and firing synchrony. Quinidine resulted in a dose-dependent reduction in mean firing rate and synchrony of KCNT1 (P924L) GABANeuron cultures.

Figure 5: Quinidine Attenuates Activity in KCNT1 (P924L) iCell GABANeurons

Figure 6: Co-culture with iCell GlutaNeurons Enhances KCNT1 (P924L) GABANeuron Phenotype

Co-culture with iCell GlutaNeurons Enhances KCNT1 (P924L) GABANeuron Phenotype.

iCell GlutaNeurons were co-cultured with either iCell GABANeurons KCNT1 (P924L) or isogenic control iCell GABANeurons at varying ratios of GlutaNeuron:GabaNeuron. Neural activity was monitored via multielectrode array (MEA) following 19 days in culture. Activity traces show that increasing the GABANeuron ratio results in increased neural network burst frequency compared to GlutaNeurons alone. Under all co-culture ratios, KCNT1 (P942L) GABANeurons showed increased network burst frequency compared to cultures containing isogenic GABANeurons.

Enhanced Disease Phenotype in iCell GABANeurons KCNT1 (P924L) when Co-Cultured with iCell GlutaNeurons.

iCell GlutaNeurons were co-cultured with either iCell GABANeurons KCNT1 (P924A) or isogenic control iCell GABANeurons at a ratio of 1:3. Neural activity was monitored via multielectrode array (MEA) following 19 days in culture. A) Raster plots show network burst frequency is increased in KCNT1 (P924L) cultures compared to isogenic control GABANeurons or iCell GlutaNeurons in monoculture.

Figure 7: Enhanced Disease Phenotype in iCell GABANeurons KCNT1 (P924L) when Co-Cultured with iCell GlutaNeurons

Product Highlights

Applications:

  • Drug target and therapeutic screening
  • Epilepsy disease modeling
  • Seizurogenic compound testing
  • Autosomal-dominant nocturnal frontal lobe epilepsy
  • Malignant migrating partial seizures of infancy