Leverage CRISPR-Engineered, Isogenic Human Cell Models to Study Disease Mechanisms with Precision and Accelerate your Therapeutic Discovery
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With bit.bio expertise, Tebubio is able to offer CRISPR-based genome editing in human iPSCs to create precise, scalable models of human disease. By introducing or correcting disease‑causing mutations in iPSCs with isogenic cell pairs, where diseased and healthy cells differ only at a single genetic locus, you will have access to a cleaner interpretation of disease mechanisms and drug responses. These edited iPSCs are then differentiated, using opti‑ox™ platform, into highly consistent human cell types. This combination of CRISPR precision and standardized differentiation allows you to study genetic diseases in relevant human cell contexts, reduce experimental variability, and accelerate target validation and therapeutic discovery. |
ioDisease Model Cells
ioDisease Model Cells are a range of deterministically programmed human iPSC-derived cells engineered to contain disease-related mutations. Each ioDisease Model Cell can be paired with a genetically matched ioWild Type Cell, to help you confidently link genotype to phenotype and make true comparisons in your data.
| Name | Reference | Description | Application |
|---|---|---|---|
| ioAstrocytes | ioEA1093 | iPSC-derived astrocytes demonstrate expected stellate morphology, express key astrocytic markers (SOX9, EAAT1, S100B and Vimentin), are capable of phagocytosis, cytokine secretion, and modulation of neuronal activity in co-culture. | Ideal for multi-cellular in vitro modelling studies of complex CNS biology |
| ioMicroglia | io1029S / L, io1021S / L | iPSC-derived microglia express key microglia markers, including TMEM119, P2RY12, IBA1, TREM2, CX3CR1, CD11b, CD45, and CD14. ioMicroglia also display chemotaxis and can be co-cultured with ioGlutamatergic Neuronsâ„¢. | Ideal for in vitro multi-cellular neuroinflammation studies and neurodegenerative disease modeling |
| ioOligodendrocyte-like cells | io1028S | Resemble a pre-myelinating oligodendrocyte and show increased MBP expression in co-culture with neurons. Express MBP, PLP1, CNP and MAG. | Ideal for compound screening and phenotypic assays for neurodegenerative and demyelinating disease |
| ioSkeletal Myocytes | io1002S / L | Contract in 2D culture in response to stimuli. 3D muscle bundles form in 3-5 days and remain stable for at least 21 days. | Ideal for skeletal muscle studies and modelling Duchenne muscular dystrophy |
| ioGABAergic Neurons | io1003S | Express GAD1, GAD2, VGAT, DLX1 and DLX2. Show spontaneous activity via calcium imaging. | Ideal for multi-cellular CNS modelling studies |
| ioGlutamatergic Neurons | io1001S / L | Express >80% glutamate transporter genes VGLUT1 and VGLUT2. Functional excitatory neurons. | Ideal for studying excitatory signalling and neurodegenerative diseases |
| ioSensory Neurons | io1024S | Highly pure sensory neuronal population (>99%) with nociceptor identity. Respond to TRPV1, TRPM3 and TRPM8 agonists. | Ideal for chronic pain research and drug development |
| ioMotor Neurons | io1027S | Express MNX1(HB9), FOXP1, ISL2 and cholinergic markers CHAT & SLC18A3. | Ideal for ALS and neuromuscular disease studies |
Alzheimer's Disease (AD)
1. Mutated ioMicroglia
Microglia are resident macrophages in the central nervous system, playing a multifunctional role in the pathogenesis of AD, where they cluster around amyloid-β deposits.
| Name | Reference | Description |
|---|---|---|
| ioMicroglia APOE 4/4 C112R/C112R (CL69) | io1032S |
The APOE4 allele is the strongest genetic risk factor for late-onset Alzheimer's disease.
Microglia that express the APOE4 gene cannot metabolize lipids normally. Free fatty molecules in the environment bind to a specific type of potassium channel embedded in neuron cell membranes, which suppresses neuron firing as well as contributing to an inflammatory burden. |
| ioMicroglia APOE 4/3 C112R/WT (CL53) | io1033S | |
| ioMicroglia TREM2 R47H/R47H (CL17) | io1035S |
The TREM2 R47H mutation has been linked with increased risk of late-onset Alzheimer's disease (AD).
TREM2, a microglial immunoreceptor, has been shown to play critical roles in synaptic development and clearance of debris. This gene is poorly conserved between mouse and human, raising the need to study its function in human models. |
| ioMicroglia TREM2 R47H/WT (CL86) | io1038S |
2. Mutated ioGlutamatergic Neurons
Glutamatergic neurons have dual role in AD: their activity is compromised due to the destruction of synapse and neuronal death, and the pathological accumulation of glutamate can induce neurotoxicity due to time-related exposure.
| Name | Reference | Description |
|---|---|---|
| ioGlutamatergic Neurons APP KM670/671NL/KM670/671NL (CLH12) | io1059S |
This in vitro disease cell model recapitulates an overall increase in the production of amyloid beta peptides, as observed in AD.
This Swedish mutation was reported to disrupt both the anterograde and retrograde axonal transport machinery, impairing the movement of multiple vesicles within axons, including APP-loaded vesicles, a subset of early endosomes, and lysosomes. (source) |
| ioGlutamatergic Neurons APP KM670/671NL/WT (CLE4) | io1061S | |
| ioGlutamatergic Neurons APP V717I/V717I (CL27) | io1063S |
This in vitro disease cell model recapitulates an increased ratio of amyloid beta peptides Aβ42:40, as observed in AD.
This mutation appears to be one of the most common APP mutations worldwide, and in addition to increasing Aβ42 levels, it alters APP subcellular localization and tau expression and phosphorylation. (source) |
| ioGlutamatergic Neurons APP V717I/WT (CL35) | io1067S | |
| ioGlutamatergic Neurons PSEN1 M146L/M146L (CL15) | io1069S | The M146L mutation in the PS1 gene (PSEN1) leads to an autosomal dominant form of early-onset AD by promoting a relative increase in the generation of the more aggregation-prone Aβ42. |
| ioGlutamatergic Neurons PSEN1 M146L/WT (CL8) | io1072S |
3. Mutated ioGABAergic Neurons
Due to contradictory evidence, researchers have recently conducted a meta-analysis to investigate whether the GABAergic system is altered in AD patients compared to healthy controls. They have concluded that GABAergic system is vulnerable to AD pathology and should be considered a potential target for developing pharmacological strategies and novel AD biomarkers.
| Name | Reference | Description |
|---|---|---|
| ioGABAergic Neurons APP V717I/V717I (CL59) | io1081S |
This mutation is linked to familial early-onset Alzheimer's disease (AD).
It is one of the most common APP mutations worldwide and increases Aβ42 levels, while also altering APP subcellular localization and tau expression and phosphorylation.
(source) |
| ioGABAergic Neurons APP V717I/V717I (CL70) | io1082S | |
| ioGABAergic Neurons APP V717I/WT (CL65) | io1084S | |
| ioGABAergic Neurons APP V717I/WT (CL54) | io1085S |
Amyotrophic Lateral Sclerosis (ALS) and Frontotemporal Dementia (FTD)
ALS and FTD are understood as part of a single neurodegenerative continuum, often called the ALS–FTD spectrum.
1. Mutated ioMotor Neurons
Motor neurons are specialized nerve cells responsible for transmitting signals from the central nervous system to muscles, enabling voluntary movement, which selectively die in ALS.
| Name | Reference | Description |
|---|---|---|
| ioMotor Neurons SOD1 G93A/G93A (CL11) | io1041S |
SOD1 was one of the first genes to be implicated in ALS-FTD and its mutation causes aggregation, toxicity and oxidative damage.
Additionally, its expression is regulated by FUS, reinforcing the importance of targeting SOD1 mRNA to limit the expression of the mutant protein without affecting the host DNA. |
| ioMotor Neurons SOD1 G93A/WT (CL25) | io1042S | |
| ioMotor Neurons TDP-43 M337V/M337V (CL84) | io1046S |
The protein TDP-43 moves from its normal location (the nucleus) to the cytoplasm and disrupts normal cellular function in ALS and FTD neurons.
This highlights it as a promising therapeutic target to slow or prevent disease progression. |
| ioMotor Neurons TDP-43 M337V/WT | io1050S | |
| ioMotor Neurons TDP-43 N352S/WT | io6017S | |
| ioMotor Neurons TDP-43 A382T/WT | io6019S | |
| ioMotor Neurons FUS P525L/P525L (CL4) | io1052S | FUS protein aggregates and behaves in a prion-like manner, spreading disease within the brain and exacerbating neurodegeneration in ALS and FTD. |
| ioMotor Neurons FUS P525L/WT (CL71) | io1055S |
2. Mutated ioGlutamatergic Neurons
The impaired uptake of glutamate released by glutamatergic neurons leads to toxicity, contributing to motor neuron degeneration.
| Name | Reference | Description |
|---|---|---|
| ioGlutamatergic Neurons TDP-43 M337V/M337V | ioEA1005L, ioEA1005S |
In cortical glutamatergic neurons, TDP-43 loss disrupts the normal splicing of the KCNQ2 potassium channel, which helps regulate neuronal excitability.
This disruption leads to intrinsic hyperexcitability, increasing the risk of mortality and underscoring its clinical significance. |
| ioGlutamatergic Neurons TDP-43 M337V/WT | ioEA1006L, ioEA1006S |
Gaucher (GD) and Parkinson’s Disease (PD)
| Name | Reference | Description |
|---|---|---|
| ioGlutamatergic Neurons GBA Null/R159W | io1007S |
Variants in GBA1, the gene encoding the lysosomal enzyme glucocerebrosidase, are the most common known genetic risk factor for Parkinson’s disease.
The null mutation of GBA increases the risk of developing Parkinson’s disease. |
Parkinson’s Disease
| Name | Reference | Description |
|---|---|---|
| ioGlutamatergic Neurons SNCA A53T/A53T (clone H5) | io1088S | The A53T mutation in the SNCA gene (encoding the α-synuclein protein involved in membrane binding, synaptic vesicle recycling, and dopamine metabolism) is one of the most significant genetic risk factors for early-onset Parkinson’s disease. |
| ioGlutamatergic Neurons SNCA A53T/WT (621P2D1) | io6005S |
Neuroinflammation Research
| Name | Reference | Description |
|---|---|---|
| ioMicroglia P2RY12 null/null (747P1F1) | io6012S |
In response to ATP release from damaged cells, P2RY12 stimulation triggers the extension of microglial processes toward the site of injury.
This chemotactic response is essential for clearing infected cells or cellular debris and plays a key role in tissue repair. |
| ioMicroglia P2RY12 null/WT (747P1D1) | io6015S |
Huntington’s Disease
| Name | Reference | Description |
|---|---|---|
| ioGlutamatergic Neurons HTT 50CAG/WT | ioEA1004L, ioEA1004S |
Expansion of CAG repeats in the HTT gene leads to the production of mutant huntingtin protein (mHTT), which is highly expressed in neurons.
This impairs the ubiquitin–proteasome system, resulting in defective proteolytic clearance and progressive accumulation of neurotoxic protein aggregates, ultimately contributing to mitochondrial dysfunction and neurodegeneration. |
Duchenne Muscular Dystrophy (DMD) Disease (DMD)
Mutated Skeletal Myocytes
Skeletal myocytes rely on dystrophin to maintain the structural integrity of their cell membrane (sarcolemma) during muscle contraction. In DMD disease, dystrophin is not functional due to DMD gene mutation resulting in progressive muscle weakness. The DMD gene is the largest gene in the human genome (79 exons), and deletions of one or more exons are the most common mutation type underlying DMD. Find below the existing options Tebubio is able to offer:
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References
Article content created by Tebubio using courtesy materials provided by bit.bio.
Figure 3: Stability of the luminescence signal of the One-Step™ Luciferase Assay.
Figure 4: Performance comparison of the One-Step™ Luciferase Assay with a leading competitor.
Why Researchers Choose the One-Step™ Luciferase Assay
Looking for Dual-Reporter Assays?
High sensitivity
Detect even low levels of firefly luciferase activity with reliable signal intensity, supporting precise quantification across a wide dynamic range.
Long signal stability
Luminescence remains stable for more than two hours, providing flexibility in plate reading and experimental scheduling.
Simple one-step protocol
The homogeneous workflow reduces handling steps, minimises pipetting errors, and improves reproducibility.
Ideal for high-throughput screening
The simplified assay format is well-suited for HTS applications, enabling efficient screening of compounds or pathway modulators.
Broad compatibility
The assay performs well in commonly used culture media containing 0–10% serum and phenol red, allowing easy integration into existing experimental protocols.
No injector required
The assay does not require a luminometer equipped with injectors, making it compatible with standard plate readers.
For experiments requiring internal normalisation or dual-reporter measurements, BPS Bioscience also offers the TWO-Step Luciferase (Firefly & Renilla) Assay.
This assay enables rapid, high-throughput quantification of both Firefly and Renilla luciferases from a single sample in mammalian cell culture, providing robust data normalisation for reporter assays.
Figure 5: TWO-Step Luciferase (Firefly & Renilla) Assay System protocol overview.
The Firefly Luciferase Reagent is added directly to the cell culture medium. This reagent lyses the cells and contains a substrate that allows Firefly luciferase to generate a luminescence signal. Next, the Renilla Luciferase Reagent is added to the same well. The reagent quenches the Firefly luciferase luminescence and provides the substrate for Renilla luciferase to generate Renilla luciferase luminescence signal. The signal generated by both reactions can be conveniently measured on a luminometer.
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References
Article content created by Tebubio using courtesy materials provided by BPS Bioscience.
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