Alexander Disease Cell Model Products
Introduction
A rare neurodegenerative disorder, Alexander Disease (AxD) is characterized by its progressive and lethal course, for which no curative treatment presently exists. Caused by dominant gain-of-function mutations in the gene encoding Glial Fibrillary Acidic Protein (GFAP), the disease is pathologically defined by the accumulation of GFAP-positive protein aggregates, known as Rosenthal fibers, within astrocytes. Studying this complex astrocytopathy has been historically challenging, hindered by the lack of cellular models that faithfully recapitulate the key hallmarks of the disease.
At Creative Biolabs, we recognize that breakthrough discoveries are built on superior models. We have dedicated our expertise in stem cell biology and genetic engineering to develop a portfolio of Alexander Disease cell models that provide researchers with the physiologically relevant, reliable, and scalable tools needed to accelerate the path toward effective therapeutics. Contact Us for a Quote.
Our product catalog is available in the 'Product List' section.
Types
We offer a flexible range of product formats to meet your specific experimental needs:
| Types | Description |
|---|---|
| iPSC-derived Astrocytes | Receive fully differentiated and characterized astrocytes harboring specific AxD mutations and their corresponding isogenic controls. These cells are ideal for studying the cell-autonomous effects of the disease. |
| Alexander Disease iPSCs | Purchase patient-derived or CRISPR-engineered iPSCs to differentiate into your specific neural cell types of interest, allowing for customized research applications. |
| Engineered Reporter Cell Lines | Our custom service builds cell lines with integrated fluorescent biosensors, enabling the live, dynamic observation of core disease processes such as stress pathway activation and GFAP aggregation. |
| Comprehensive Service Packages | Partner with us for end-to-end support, from model generation and characterization to assay development and screening. |
Advantages
Choosing the right model is critical. Our AxD models provide a clear advantage, enabling higher quality, more predictive data in less time. Leverage these core benefits for your research.
True-to-Disease Human Physiology
Our human iPSC models offer true-to-disease pathology by expressing endogenous mutant GFAP, leading to spontaneous protein aggregation and cellular stress responses. This allows you to study the direct impact of GFAP mutations in a physiologically relevant system, avoiding the artifacts common to artificial overexpression models.
Unambiguous, High-Confidence Data
Utilize our CRISPR-engineered isogenic controls to eliminate confounding genetic variability. This unparalleled precision enables the confident identification of disease-specific molecular pathways and therapeutic targets by perfectly matching diseased cells to their corrected counterparts.
Dramatically Accelerated Research Timelines
Save valuable time and resources with our ready-to-use, fully characterized cell models. Bypass the complex and lengthy process of in-house model development and accelerate your research, moving from experimental setup to high-quality data generation in days, not months.
A True Scientific Partnership
We provide more than a product; we offer a partnership. Benefit from the expert guidance of our PhD-level scientific team throughout your project—from initial model selection to final data interpretation—ensuring your unique research succeeds.
Broad Applications
Creative Biolabs' TSC cell models are versatile tools suitable for a wide range of research applications:
| Applications | Description |
|---|---|
| Investigate Disease Mechanisms | Elucidate the downstream consequences of mutant GFAP expression, including its effects on protein folding and degradation, cytoskeletal integrity, and astrocyte function. |
| High-Throughput Drug Screening | Screen small molecule libraries to identify compounds that can ameliorate AxD-related phenotypes, such as reducing GFAP aggregation or restoring normal astrocyte function. |
| Target Identification and Validation | Use our models to validate novel therapeutic targets and to understand the mechanism of action of lead compounds. |
| Biomarker Discovery | Identify and validate soluble or cell-based biomarkers that can be used for disease diagnosis, prognosis, or monitoring treatment response. |
| Personalized Medicine Approaches | Explore how different GFAP mutations influence disease severity and response to potential therapies using our panel of patient-derived models. |
Fig.1 Phenotype, defective cellular processes, and available pathological models of Alexander diseases.1
FAQs
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Can I request a model with a specific GFAP mutation that isn't listed?
Absolutely. Our custom services team specializes in generating models for rare or novel mutations. We invite you to contact us to discuss the specifics of your desired variant, and we can provide a detailed project proposal. -
What kind of support do you provide after purchase?
We provide comprehensive support. Every purchase comes with detailed protocols and access to our scientific support team. They can help you with everything from initial cell culture troubleshooting to optimizing your experimental setup for the best results. -
How long does it take to develop a custom model?
The timeline for custom model creation is tailored to the scope of each project. We invite you to contact us for a comprehensive proposal and a precise timeline. -
Are these cells for in vivo use?
No, these cells are intended for in vitro research use only and are not for human or animal therapeutic use. -
How do I know which model is right for my research?
Our expert scientific team can help you choose the best model for your specific research question and experimental setup. Please contact us to schedule a free consultation.
Creative Biolabs provides the highest quality, most physiologically relevant Alexander Disease cell models available, backed by expert support and a commitment to scientific excellence. We empower you to ask bigger questions and get clearer answers, faster. Our team of scientists is ready to collaborate with you to adapt our products for your specific research objectives. For pricing, technical details, or to start a custom project, please reach out.
Related Product Sections
The following links provide access to additional research tools for Rare & Metabolic Diseases.
Reference
- Lanciotti, Angela, et al. "Human iPSC-derived astrocytes: A powerful tool to study primary astrocyte dysfunction in the pathogenesis of rare leukodystrophies." International Journal of Molecular Sciences 23.1 (2021): 274. DOI: 10.3390/ijms23010274. Use under Open Access license CC BY 4.0, without modification.