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Charcot-Marie-Tooth Disease (CMT) Cell Model Products

Introduction Types Advantages Applications FAQs Related Product Sections Product List

Introduction

Charcot-Marie-Tooth (CMT) disease's profound genetic heterogeneity presents a formidable challenge, often unmet by conventional lab models that lack patient-specific mutations. Creative Biolabs confronts this gap with an advanced portfolio of CMT cell models. Derived from patient iPSCs and precision-engineered, these physiologically pertinent platforms provide a robust system to dissect elusive disease mechanisms and confidently screen therapeutic candidates. Our models are engineered to accelerate the translational pipeline, moving research from discovery to preclinical validation with greater speed and precision. Let our specialists help you select or create the optimal model to advance your investigation. Contact an Expert Today.

For a detailed enumeration of our specific offerings, please consult our comprehensive Product List.

Creative Biolabs' Comprehensive Portfolio of CMT Cell Models

We offer an extensive catalog of ready-to-use and custom-developed CMT cell models, designed to meet the diverse needs of your research.

Types Description
Patient-Derived "Disease-in-a-Dish" Models Our portfolio is built on iPSC models sourced from patients with confirmed CMT. By reprogramming their somatic cells, we create a platform that authentically carries the patient's unique genetic signature, enabling the study of mutation-specific dysfunctions.
Differentiated Neuronal Lines We differentiate these iPSCs into motor neurons to study axonal defects (crucial for CMT2) and sensory neurons to investigate the prominent sensory deficits of the disease, such as pain and numbness.
Specialized Schwann Cells Our iPSC-derived Schwann cells are indispensable for modeling demyelinating forms like CMT1 and CMT4, allowing for detailed analysis of myelination defects and key protein interactions.
Broad Subtype Coverage Our catalog includes models for CMT1A (PMP22), CMT1B (MPZ), CMTX1 (GJB1), CMT2A (MFN2), and other rare variants.
Isogenic Cell Lines for Precision For ultimate experimental control, we use CRISPR/Cas9 to engineer isogenic cell line pairs.
Mutation Correction We can correct a disease-causing mutation in a patient's cell line to create a genetically identical control, definitively linking a phenotype to that specific mutation.
Mutation Introduction Conversely, we introduce known CMT mutations into healthy cell lines to study the functional consequences of a single variant.

Advantages: Your Competitive Edge in CMT Research

Choosing Creative Biolabs' CMT models translates features into tangible benefits that accelerate your research and increase your probability of success.

Enhance Predictive Accuracy

By using human cells with endogenous disease mutations, you can more accurately predict how a therapeutic candidate will perform in patients, reducing the risk of late-stage clinical failures.

Shorten Research Timelines

Skip the time-consuming and often unpredictable process of model development. Our assay-ready, validated cells are prepared for immediate use in your screening and validation workflows.

Achieve Consistent & Reproducible Data

Eliminate the variability of primary tissue samples and less characterized models. Our stringently quality-controlled cell lines provide a stable, reproducible system for generating reliable data.

Uncover Novel Disease Insights

Our physiologically relevant models, including co-culture and 3D spheroid systems, allow you to investigate complex cellular interactions, myelination defects, and axonal transport issues that are central to CMT pathology.

Cost-Effective & Ethical Research

Reduce reliance on complex and expensive animal models for initial screening. Our in vitro models offer a powerful, scalable, and ethically sound platform for high-throughput discovery.

Applications: Powering Every Stage of Drug Discovery

Our CMT cell models are versatile tools designed to support a wide range of applications across the research and development pipeline.

Applications Description
High-Throughput Screening (HTS) Screen small molecule libraries to identify compounds that rescue disease phenotypes.
Mechanism of Action (MoA) Studies Elucidate the precise molecular pathways disrupted by CMT mutations.
Novel Target Identification & Validation Use our models to uncover new, druggable targets for intervention.
Gene & Cell Therapy Development Provide a critical in vitro platform to test the efficacy and safety of AAV-based gene therapies or other advanced therapeutic modalities.
Personalized Medicine Approaches Assess the potential efficacy of a compound on cells derived from patients with specific genetic profiles.
A picture that presents Model for how CMT mutations in INF2 reduce mitochondrial mobility. (Schiavon, et al., 2021) (OA Literature)Fig.1 Model for how CMT mutations in INF2 reduce mitochondrial mobility.1

FAQs

  • What types of cells are most relevant for CMT models?
    The most critical cell types are Schwann cells (which produce the myelin sheath) and various types of neurons (motor and sensory). Our models focus on these and can be provided in monocultures or co-culture systems.
  • What if I need a model for a rare CMT mutation?
    We specialize in custom model development. Our scientific team can work with you to create a new iPSC line or use gene editing (like CRISPR/Cas9) to introduce a specific mutation into a healthy control line.
  • Do you provide differentiated cells or just iPSCs?
    We provide both. You can purchase the undifferentiated iPSCs, or you can order vials of our expertly differentiated, cryopreserved, and assay-ready Schwann cells or neurons.
  • Do you provide technical support?
    Yes, all our products come with dedicated technical support from the same scientists who develop and validate the models. We are here to help you succeed.
  • How are the cells shipped?
    Cells are shipped frozen on dry ice via a priority courier to ensure their viability upon arrival.

Advancing therapies for a disease as complex as Charcot-Marie-Tooth requires the best tools. Creative Biolabs provides more than just cell lines; we offer a partnership. By leveraging our deeply characterized and clinically relevant CMT models, you can de-risk your programs, accelerate your timeline, and focus on what matters most: bringing a life-changing treatment to patients.

Ready to discuss your specific needs? Contact our neuroscience team today for a free consultation and to learn how our models can power your next discovery.

Related Product Sections

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Reference

  1. Schiavon, Cara R., Gerald S. Shadel, and Uri Manor. "Impaired mitochondrial mobility in Charcot-Marie-Tooth disease." Frontiers in Cell and Developmental Biology 9 (2021): 624823. DOI: 10.3389/fcell.2021.624823. Use under Open Access license CC BY 4.0, without modification.