Axol Bioscience Ltd.

Name: Axol Bioscience Ltd.
Address: Cambridge, CB101XL, GB
Telephone: +44(0)1223 751 051

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The Best Human Cell Culture Systems for Research Success Contact Axol Bioscience today! www.axolbio.com

Axol offer ready-made, well-characterized stem and functional cells complete with easy-to-understand protocols and video guides for handling them. We are devoted to making high quality, data-rich cellular products that are affordable for all research labs including starting up labs! We believe that all research labs should have easy access to the cells generated using the reprogramming and downstream differentiation technologies.

21/10/2026

Friedreich's Ataxia in vitro model using patient iPSC-derived cardiomyocytes

The paper by Less G.J., and colleagues investigates the mechanisms underlying cardiomyopathy in Friedreich ataxia (FRDA), a disorder caused by GAA repeat expansions in the FXN gene that reduce levels of the mitochondrial protein frataxin.

Using iPSC-derived cardiomyocytes from three FRDA patients and genetically matched CRISPR-corrected isogenic controls, the authors showed that frataxin deficiency causes intrinsic cardiac abnormalities, including impaired contractility, prolonged contraction and relaxation times, irregular calcium handling, increased cardiomyocyte size, elevated mitochondrial reactive oxygen species (ROS), mitochondrial fragmentation, and enhanced cell death.

Moreover, iron supplementation exacerbated these defects, highlighting the vulnerability of frataxin-deficient cells to metabolic stress, and transcriptomic analysis identified a distinct FRDA signature, with consistent upregulation of MEG3 and PCDHGA10 across all patient lines. Functional studies demonstrated that silencing either gene significantly reduced cardiomyocyte death, while PCDHGA10 knockdown also normalized mitochondrial ROS levels.

The study concludes that frataxin deficiency drives early cardiac pathology and identifies MEG3 and PCDHGA10 as promising therapeutic targets for FRDA-associated cardiomyopathy.

Read the full article: https://hubs.la/Q04BmTKJ0

Learn more about our cardiovascular offerings: https://hubs.la/Q04BmTd40 and explore our iPSC lines library: https://hubs.la/Q04BmW530

14/10/2026

Partial in vitro rescue of NPHP5-associated retinal ciliopathy via AAV-mediated delivery

The authors found that patient cells exhibited abnormally elongated primary cilia, reduced expression of CEP290, a key transition-zone protein that interacts with NPHP5, and evidence of defective ciliary trafficking. In retinal organoids, photoreceptors developed normally at early stages but later showed impaired outer-segment formation, diminished connecting cilia, and mislocalization of photoreceptor proteins, including rhodopsin and cone opsins, indicating defective photoreceptor maturation and function. Importantly, adeno-associated virus (AAV)-mediated delivery of a normal IQCB1/NPHP5 gene partially restored outer-segment development, corrected protein mislocalization, and increased CEP290 levels.

The findings suggest that NPHP5 is required for proper ciliary gate function and photoreceptor outer-segment biogenesis. Overall, the study establishes a robust human retinal organoid model of NPHP5-LCA and provides strong preclinical evidence supporting gene augmentation therapy as a potential treatment.

Read the full article: https://hubs.la/Q04B60cc0

Learn more about our solutions for retinal disease modeling & safety assessment: https://hubs.la/Q04B60cW0

09/10/2026

Building complexity into human iPSC-derived disease models

Neurodegenerative and retinal diseases involve complex interactions between neurons, glial cells and immune populations. Understanding these cell-cell interactions is important for developing physiologically relevant in vitro models that better reflect human disease biology.

In our new whitepaper, we examine how human iPSC-derived co-culture systems can be used to investigate disease-relevant cellular crosstalk while balancing biological complexity with experimental robustness and reproducibility.

Using ALS and dry age-related macular degeneration (dry AMD) as examples, we discuss:

• The role of microglia and astrocytes in neuroinflammation and disease progression
• Practical considerations for establishing complex co-culture systems
• How the addition of microglia and astrocytes alters motor neuron activity in an ALS tri-culture model
• How interactions between microglia and RPE cells influence inflammatory responses and sensitivity to retinal stress
• Emerging approaches for developing increasingly complex physiologically relevant disease models

Download the whitepaper to learn how human iPSC-derived co-culture systems can reveal disease-relevant phenotypes and provide valuable insights into cellular interactions in neuroscience and ocular research.

https://hubs.la/Q04zKK_50

07/10/2026

Tau protein aggregation is a hallmark of Alzheimer's disease and other tauopathies, yet little is known about where tau is translated and how newly synthesized tau is regulated within neurons. Using STARFISH, an innovative method for imaging endogenous mRNA translation at near-codon resolution, Konrad-Vicario and colleagues discovered that dendritically synthesized tau is continuously monitored by neuroproteasomes, defining a previously unrecognized proteostasis mechanism that protects against tau aggregation and neurodegeneration.

Key findings

• STARFISH enabled visualization of endogenous mRNA translation with single-molecule sensitivity and near-codon spatial resolution
• Despite widespread Mapt mRNA localization, tau translation occurred exclusively within neuronal dendrites
• Approximately 30% of newly synthesized tau was rapidly degraded by neuron-specific neuroproteasomes
• Neuroproteasome-mediated clearance acted as a quality control mechanism for newly translated tau
• Neuroproteasome inhibition resulted in protein synthesis-dependent accumulation of phosphorylated, mislocalized, and aggregated endogenous tau
• The findings identify a previously unrecognized proteostasis pathway that may protect neurons from tau aggregation and Alzheimer's disease-associated pathology

These findings provide new insight into the spatial regulation of tau biology and suggest that failure of neuroproteasome-mediated quality control may contribute to the development of tau pathology. The study also highlights the potential of STARFISH as a powerful tool for investigating localized translation, proteostasis, and disease-associated mechanisms in human neurons.

Read the full paper: https://hubs.la/Q04z0jsv0

At Axol Bioscience, we support tau and Alzheimer's disease research with human iPSC-derived neuronal and glial models for studying proteostasis, neuroinflammation and neurodegeneration.

Explore our Alzheimer's disease APOE portfolio: https://hubs.la/Q04z0jp00

Contact us at [email protected]

02/10/2026

We'll be at ELRIG Drug Discovery 2026 on 14-15 October, in London

Our team will be at booth M02 and ready to discuss physiologically relevant human iPSC-derived models for drug discovery.

Marking 20 years of ELRIG Drug Discovery, this year’s programme will explore developments across the field, from multiomics and next-generation biophysics to AI in compound discovery and experimental design.

Our research team will be presenting three posters:

1. Characterization of human iPSC-derived motor neuron disease model for ALS drug discovery
2. New Approach Methodologies (NAMs): assessing cardiotoxicity and neurotoxicity in vitro using human iPSC-derived cells
3. Establishing a robust platform for investigating the pro- and anti-inflammatory response of iPSC-derived microglia in drug discovery

Meet Liam Taylor, Mariana Argenziano, Sapna Vyas, Gizem Inak, Yanis Kasioulis, Louise Watts, Lewis Walker and Michael Chan at booth M02 and explore how we can support your research.

Contact us at [email protected]

Download our catalog: https://hubs.la/Q04z1nDz0

01/10/2026

We'll be at the UK Organ-On-A-Chip (OOAC) annual symposium on 7 October in London

Our Scientist, Ellie Bravington, will be there to connect with researchers and leaders in the field.

The symposium brings together the community working across preclinical translational models, including organ-on-a-chip, organoids, spheroids, NAMs and MPS.

Ellie will be presenting our poster in collaboration with Optics11 Life and King's College London: "A highly reproducible and precise measurement platform for a 3D engineered cardiac muscle tissue contractility"

Key highlights

- 3D engineered heart tissues showed stable maturation and functionality for up to 42 days, with increased spontaneous contractile force and contraction/relaxation velocity
- Isoprenaline challenge under 1.5 Hz paced conditions resulted in a dose-dependent increase in force of contraction, demonstrating sensitivity to drug-induced functional changes
- The platform combines axoCells™ ventricular cardiomyocytes with the Cuore contractility system for real-time force measurement with sub-micronewton precision and long-term assessment of 3D engineered cardiac tissues

Download the poster: https://hubs.la/Q04yTkw-0

If you’re attending, come and meet Ellie to discuss how physiologically relevant in vitro models can support your research.

30/09/2026

Huntington's disease (HD) is caused by expansion of the HTT CAG repeat, but the role of somatic repeat expansion in disease progression remains an active area of research. Chris Kay and colleagues showed that loss of interruption (LOI) within the expanded HTT CAG repeat increases somatic expansion in medium spiny neurons (MSNs), accelerating striatal pathology and disease progression.

Key findings

• Large (111-150 CAG) and very large (>150 CAG) expansions accumulate in HD-affected MSNs
• The CAG-CCG LOI modifier increased large somatic expansions in MSNs by ~5-fold
• LOI carriers showed reduced caudate MSN counts, linking somatic expansion to neuronal loss
• Increased somatic CAG expansion was associated with earlier and more severe HD pathology
• The findings support somatic repeat expansion as a key driver of striatal neurodegeneration in HD

These findings highlight somatic instability as a potential disease-modifying mechanism in Huntington's disease and reinforce the value of human MSN models for translational research and drug discovery.

Read the full paper: https://hubs.la/Q04yHLkM0

At Axol Bioscience, we support Huntington's disease research with human iPSC-derived ax0219 HTT CAG142/143 striatal neurons (https://hubs.la/Q04yHKF70), enabling researchers to investigate somatic instability, mutant huntingtin biology, and MSN vulnerability in a patient-derived model. We also offer assay services, including CAG repeat sizing, mHTT analysis, MEA recordings, and neurodegeneration studies to support therapeutic development.

Explore our Huntington's disease poster: https://hubs.la/Q04yHKF80

Contact us at [email protected]

23/09/2026

Characterizing new variants of late-onset retinal dystrophy

In this study, the researchers identified SAXO6 (formerly MDM1) as a novel gene associated with inherited retinal dystrophy. Genetic analysis of six affected individuals from five unrelated families revealed six different biallelic loss-of-function SAXO6 variants, associated with late-onset retinital dystrophy. Visual symptoms typically emerged relatively late, with a median onset of 44 years, followed by progressive retinal degeneration.

Using high-resolution expansion microscopy and immunogold electron microscopy, the researchers showed that SAXO6 localizes along microtubules in the cilia of human rod and cone photoreceptors, as well as in motile airway cilia. Cross-linking mass spectrometry further demonstrated an interaction between SAXO6 and α-tubulin, supporting its classification as a microtubule inner protein (MIP).

The authors conclude that SAXO6 likely contributes to ciliary microtubule stability and long-term photoreceptor maintenance, establishing SAXO6-associated retinal degeneration as a ciliopathy and highlighting MIPs as an underexplored class of proteins relevant to inherited blindness.

Read the full article: https://hubs.la/Q04y692C0

Learn more about our solutions for retinal disease modeling & safety assessment : https://hubs.la/Q04y68Zk0

21/09/2026

New human iPSC-derived co-culture systems for more physiologically relevant ALS modeling

Human iPSC-derived co-culture systems incorporating motor neurons, astrocytes and microglia provide a more physiologically relevant approach for studying neuroinflammation, cellular crosstalk and disease mechanisms.

Our new portfolio provides complete, standardized co-culture solutions for investigating the contribution of glial cells to ALS and other neurodegenerative disorders.

The portfolio includes:

• axoCells™ human iPSC-derived motor neurons + microglia + astrocytes co-culture (unaffected) cells, media and supplement kit

• axoCells™ human iPSC-derived motor neurons + astrocytes co-culture (unaffected) cells, media and supplement kit

• axoCells™ human iPSC-derived motor neurons + microglia co-culture (unaffected) cells, media and supplement kit

• axoCells™ human iPSC-derived motor neuron media and supplement co-culture kit

Our data demonstrate that glial cells can influence motor neuron network activity, highlighting the importance of cellular context when modelling ALS.

Additional motor neurons, astrocytes and microglia can also be generated through our custom differentiation services.

Learn more and explore the new kits: https://hubs.la/Q04xXKhl0

16/09/2026

Mesenchymal drift in ciliopathy iPSC-derived RPE reveals a convergent pathogenic cell state

In this study, Dominik Reichert and colleagues used induced pluripotent stem cell-derived retinal pigment epithelium (iPSC-derived RPE) cells from nine patients carrying mutations in seven ciliopathy genes (BBS1, BBS10, BBS16, CEP290, LCA5, MYO7A, and PRPF31) to identify shared pathogenic mechanisms underlying retinal degeneration.

Despite diverse genetic defects and variable ciliary abnormalities, all patient-derived RPE cells exhibited a common disease state characterized by loss of epithelial polarization, altered cell morphology, and a shift toward a mesenchymal-like phenotype ("mesenchymal drift"). Transcriptomic, structural, and functional analyses revealed widespread mitochondrial dysfunction and constitutive activation of TGF-β signaling as convergent pathological drivers. The authors showed that these defects occur downstream of primary cilium dysfunction and are largely mutation-independent.

A targeted phenotypic drug screen identified two clinically relevant compounds, pioglitazone and galunisertib, which partially restored RPE morphology, polarity, mitochondrial function, and gene expression across multiple ciliopathy subtypes.

The study concludes that targeting shared downstream pathways may provide broadly applicable therapies for genetically diverse ciliopathies.

Read the full paper: https://hubs.la/Q04wHlwL0

Our human iPSC-derived RPE cells express the key markers (MITF, ZO-1 and PMEL17) and display typical morphology. That means robust, physiologically relevant models of the retina, for ocular research and drug discovery.

Learn more: https://hubs.la/Q04wHlwb0

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Cambridge
CB101XL

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+44(0)1223 751 051

Website

http://www.axolbio.com/

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