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Leukodystrophies Cell Model Products

Introduction Types Advantages Applications FAQs Related Product Sections Product List

Introduction

Leukodystrophies are a group of rare, progressive genetic disorders that affect the white matter of the central and peripheral nervous systems. The underlying pathology often involves the dysfunction or loss of myelin-producing glial cells, leading to devastating neurological decline. Studying these complex diseases requires physiologically relevant models that can accurately recapitulate human disease mechanisms.

At Creative Biolabs, we provide researchers with a comprehensive portfolio of high-purity, patient-derived, and expertly engineered cell models for a wide range of leukodystrophies. Our models empower the scientific community to dissect disease pathways, identify novel therapeutic targets, and accelerate the development of life-changing treatments. Consult with our experts to identify the optimal cell model for your research requirements.

To see all available products, navigate directly to the Product List section.

Main Types

Types Description
Patient-Derived iPSCs Pluripotent stem cells carrying mutations for diseases such as Metachromatic Leukodystrophy (MLD), Krabbe Disease, Adrenoleukodystrophy (X-ALD), Alexander Disease, and more.
Disease-Specific Astrocytes Ideal for studying astrocytopathies like Alexander Disease (GFAP mutations) and investigating the role of astrocytes in supporting or hindering myelination.
Disease-Specific Oligodendrocyte Progenitor Cells (OPCs) & Mature Oligodendrocytes Directly model defects in myelin production and maintenance. Essential for screening compounds aimed at promoting oligodendrocyte survival and differentiation.
Disease-Specific Microglia Crucial for investigating the neuroinflammatory component of leukodystrophies and the impact of microglial activation on disease progression.
Healthy Donor Control Cells Genetically normal iPSCs and differentiated glial cells from healthy donors to serve as isogenic controls (when available) or general wild-type comparators.

Advantages

Partnering with Creative Biolabs provides your research with a critical competitive edge.

High Physiological Relevance

By using human cells with endogenous mutations, our models offer a more accurate representation of disease pathology compared to traditional animal models.

Unravel Complex Glial Interactions

Study the intricate crosstalk between astrocytes, oligodendrocytes, and microglia to understand their respective roles in myelin maintenance and degradation.

Accelerate Drug Discovery

Our assay-ready models provide a consistent and scalable platform for high-throughput screening (HTS) of small molecules, biologics, and gene therapies.

Enhance Reproducibility

Reduce experimental variability with highly characterized, standardized cell populations, leading to more robust and publishable data.

Save Time and Resources

Bypass the lengthy and complex process of iPSC reprogramming, characterization, and differentiation. Our models allow your team to focus immediately on key research questions.

Access Rare Disease Models

Gain access to cellular models for ultra-rare leukodystrophies that are otherwise difficult or impossible to source.

Applications

Our Leukodystrophies models are robust, validated tools engineered for a wide array of applications, empowering you to answer critical questions at every stage of the drug discovery pipeline.

Applications Description
Disease Modeling Investigate the cellular and molecular mechanisms driving specific leukodystrophy subtypes, such as defects in myelination, lipid metabolism, and inflammatory responses.
Therapeutic Compound Screening Identify and validate novel drug candidates that can rescue disease phenotypes, promote myelin repair, or reduce neuroinflammation.
Gene Therapy Validation Test the efficacy and safety of AAV-mediated gene replacement or CRISPR-based gene editing strategies in a human-specific context.
Biomarker Discovery Analyze secreted factors, proteins, and metabolites from diseased cells to identify potential biomarkers for diagnosis or disease monitoring.
Neuroinflammation Studies Elucidate the role of microglia and astrocytes in initiating and propagating the inflammatory cascades characteristic of many leukodystrophies.
Toxicology and Safety Assessments Evaluate the potential neurotoxic effects of candidate compounds on specific neural cell types.
A picture that presents the molecular Mechanisms of Leukodystrophies. (Amodio, et al., 2022) (OA Literature)Fig.1 Therapeutic options for the treatment of autosomal recessively inherited leukodystrophies.1

FAQs

  • Can you create a model for a specific mutation that is not currently listed?
    Absolutely. Our custom development service is a core part of our offering. Our scientific team can work with you to engineer specific mutations using advanced gene-editing techniques. We encourage you to contact us to discuss the feasibility of your specific project.
  • What should I do if I have trouble culturing the cells?
    Creative Biolabs provides expert technical support. If you encounter any issues, please contact our support team immediately. We can help troubleshoot everything from thawing to downstream assay performance.
  • Can these models be used for co-culture experiments?
    Yes, they are ideal for establishing physiologically relevant co-culture systems, such as neuron-glia or astrocyte-oligodendrocyte co-cultures, to study intercellular signaling and its role in disease.
  • Do you offer cells representing different subtypes of a single leukodystrophy (e.g., infantile vs. adult MLD)?
    Our catalog is constantly expanding. We often carry models representing different clinical severities or onset forms of a disease. Please check our online catalog or inquire with our sales team for specific variants.
  • Can I publish data generated using your cells?
    Yes. We encourage publication and simply require that you cite Creative Biolabs as the source of the cell models in your materials and methods section.

The path to understanding and treating leukodystrophies is complex. Success requires the most advanced, reliable, and clinically relevant research tools. Creative Biolabs is committed to providing these tools to the dedicated scientists working on the front lines of neurological disease research. Our expertly crafted cell models remove technical barriers, enabling you to focus on what matters most: discovery. Contact us for more assistance.

Related Product Sections

Additional categories of research tools for Rare & Metabolic Diseases can be accessed via the following links.

Reference

  1. Amodio, Federica, et al. "An overview of molecular mechanisms in Leukodystrophies." Biomolecules 12.10 (2022): 1460. DOI: 10.3390/biom12101460. Use under Open Access license CC BY 4.0, without modification.