Benign Essential Blepharospasm Cell Model Products
Introduction
Benign Essential Blepharospasm (BEB) is a debilitating neurological disorder characterized by involuntary, repetitive muscle contractions of the eyelids, leading to functional blindness. As a form of focal dystonia, its precise etiology remains enigmatic, posing significant challenges for therapeutic development. At Creative Biolabs, we empower researchers and pharmaceutical innovators to overcome these hurdles. We provide the world's most advanced, well-characterized, and reliable BEB-relevant cell models, designed to de-risk your research and accelerate the journey from target identification to clinical success.
Advance your BEB research today. For detailed specifications, pricing, or to discuss a custom project, please request a quote or consult with our specialists.
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Types
We offer a selection of models to meet diverse research needs. Custom models can also be developed upon request.
| Types | Description |
|---|---|
| Donor-Derived iPSC Lines | Generated from individuals with a BEB diagnosis, these iPSCs carry the donor's unique genetic signature. They provide a renewable foundation for studying molecular mechanisms and creating differentiated models. |
| iPSC-Derived Neural and Glial Cells | We generate highly pure, functional neural and glial populations from our iPSC lines. These include Medium Spiny Neurons (MSNs), dopaminergic neurons, cortical neurons, and astrocytes. They are ideal for studying specific cell functions, neuron-glia interactions, and building complex, physiologically relevant co-culture systems for your research. |
| Neural Co-Culture Systems | Move beyond monocultures to model synaptic connectivity. We establish custom systems, such as striatal MSNs with cortical neurons, to better replicate in vivo cellular cross-talk for your research. |
| Brain Organoid Models | Our cutting-edge forebrain and midbrain organoids self-assemble into complex 3D structures. They are unparalleled for studying developmental aspects, network activity, and complex cellular interactions in a donor-specific context. |
| Genetically Engineered Isogenic Lines | Using CRISPR/Cas9, we create the perfect control by correcting genetic variants in donor iPSCs. This allows for precise investigation into the functional role of specific genes and their impact. |
Advantages
Uncompromising Quality & Reproducibility
Every cell lot is backed by a comprehensive Certificate of Analysis, confirming identity, purity, and viability. This ensures consistent, reproducible results and removes experimental uncertainty from your research program.
High Physiological Relevance
Our models are derived from human sources with relevant genetic backgrounds and differentiated into key neural types, providing a more translatable in vitro system than traditional immortalized cell lines.
Advanced System Complexity
Move beyond simple monocultures. We provide sophisticated co-culture and 3D organoid models that allow for the investigation of complex neural network dynamics and critical cell-to-cell interactions.
Precision Genetic Engineering
Utilize our CRISPR/Cas9 services to obtain perfectly matched isogenic controls or custom-engineered lines. This enables the precise validation of genetic function and dissection of molecular pathways with high confidence.
Dedicated Scientific Partnership
Our team of PhD-level scientists provides expert technical support, not just sales. We partner with you to help select the right models, optimize protocols, and achieve your research goals.
Applications
From foundational discovery to preclinical validation, Creative Biolabs models are engineered to provide actionable data across a wide spectrum of research applications.
| Applications | Description |
|---|---|
| Pathophysiology & Mechanistic Dissection | Delve into the cellular underpinnings of neuronal hyperexcitability. Perform detailed analyses of signaling pathways, protein interactions, and network dysfunction to precisely map the molecular events leading to BEB phenotypes. |
| Functional & Electrophysiological Characterization | Go beyond static observation. Utilize multi-electrode array (MEA) or patch-clamp studies to analyze neuronal excitability, synaptic transmission, and network firing patterns, directly identifying functional deficits in a human cellular context. |
| Genetic Factor Validation & Gene Function Analysis | Confirm the role of candidate genes in focal dystonias. Use our CRISPR-edited and isogenic control lines to definitively link specific genetic variants to observable cellular phenotypes with high confidence. |
| High-Throughput Therapeutic Screening (HTS) | Establish robust and scalable screening campaigns. Screen compound libraries to identify novel drug candidates that effectively modulate neuronal activity and rescue pathological phenotypes, accelerating hit-to-lead discovery. |
| Mechanism of Action (MoA) Elucidation | Investigate how novel and existing therapeutics, including botulinum neurotoxins (BoNTs), exert their effects. Use molecular probes and functional readouts to dissect the specific cellular cascades impacted by your compound. |
| Advanced Neural Network & Circuit Modeling | Reconstruct simplified neural circuits using our sophisticated co-culture and 3D organoid systems. Investigate how critical cell-to-cell interactions contribute to the overall network dysfunction seen in BEB. |
| Neurotoxicity & Safety Pharmacology | Assess the potential neurotoxic side effects of drug candidates early in the development pipeline. Evaluate effects on a sensitive and physiologically relevant human neuronal population to make confident safety decisions. |
Fig.1 The dopaminergic system in the striatum.1
FAQs
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What is included with my purchase?
Your purchase includes one vial of cryopreserved cells, a lot-specific Certificate of Analysis (CofA), and detailed protocols for thawing, plating, and maintenance. -
How do I place an order?
You can place an order through the "Request a Quote" form on our website or by emailing our sales department directly. Our team will guide you through the entire process. -
Do you provide technical support?
Yes, our team of PhD-level scientists is available to help you with any questions regarding cell handling, experimental setup, and data interpretation. This support service is included with your purchase. -
Can Creative Biolabs develop a custom cell model for me?
Yes, we offer comprehensive custom model development services, including iPSC generation from specific donor lines, gene editing, and creating tailored differentiation protocols to meet your unique research needs. -
Do you offer discounts for large-volume orders?
Yes, we provide competitive pricing for bulk orders. Please contact our sales team to discuss your volume requirements and to receive a customized quotation.
Choosing the right biological tools is critical for success. Creative Biolabs' BEB-associated cell models offer the human physiological relevance, reproducibility, and convenience your research demands. Let us handle the complexities of model development so you can focus on answering the key scientific questions. Contact us today to speak with a specialist and learn how our models can be integrated into your research pipeline.
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Reference
- Zhu, Lixia, et al. "The pathogenesis of blepharospasm." Frontiers in Neurology 14 (2024): 1336348. DOI: 10.3389/fneur.2023.1336348. Use under Open Access license CC BY 4.0, without modification.