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Prion Disease Cell Model Products

Introduction Types Advantages Applications Features FAQs Related Product Sections Product List

Introduction

Creative Biolabs is at the forefront of neurodegenerative disease research, offering an unparalleled portfolio of validated, ready-to-use prion disease cell models. We empower our clients to overcome the most significant hurdles in drug discovery, from high-throughput screening to preclinical validation, with robust tools that recapitulate the complex biology of these devastating disorders.

Prion diseases constitute a class of invariably fatal neurodegenerative disorders precipitated by the conformational conversion of the cellular prion protein (PrPC) into its pathogenic isoform (PrPSc). Therapeutic development is hindered by a critical bottleneck: distinct prion "strains" and the "species barrier" cause compounds validated in conventional models to fail against human prions. At Creative Biolabs, we break this impasse with a comprehensive suite of sophisticated and reliable cellular models, designed to address the specific, nuanced questions researchers face at every stage of the drug discovery process. Contact our experts to discuss your specific project and select the perfect model for your research. Get a quote today!

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

Types

We offer a tiered selection of models to meet every research need and budget.

Types Description
RK13 Lines Rabbit kidney cells (PrP-null) expressing ovine, elk, goat, or bank vole PrP for studying natural scrapie, CWD, and for use as a potential "universal acceptor."
PrP-Knockout Lines Mouse cell lines (e.g., CAD5, MEFs) with endogenous PrP removed via CRISPR/Cas9, providing a clean background for expressing any PrP variant of interest.
iPSC-Derived Astrocytes Human astrocytes differentiated from induced pluripotent stem cells, shown to successfully replicate CJD prions.

Advantages

Overcome the Species Barrier

Directly test your compounds against a variety of non-mouse prion diseases. We provide genetically engineered models, such as PrP-null cell lines expressing specific prion proteins from sheep, deer, or other species, allowing you to study natural scrapie or CWD in a controlled, versatile cellular environment.

Investigate Complex Biology with Precision

Our diverse portfolio enables you to probe the critical nuances of prion pathogenesis. You can effectively evaluate strain-specific therapeutic responses, a crucial factor as many compounds show different efficacy against different prion strains. Furthermore, these models are an ideal platform for studying and predicting the emergence of drug-resistant prion strains.

Expert Support at Every Step

You're not just acquiring a research tool; you're gaining a scientific partner. Our team of prion biology experts is available to help you select the most appropriate model for your project and provide support to ensure your experiments are a success.

Applications

Our prion disease cell models are the ideal platform for a wide range of research applications:

Applications Description
Primary HTS Campaigns Efficiently screen thousands of small molecules to identify novel anti-prion scaffolds.
Lead Optimization and SAR Studies Compare the potency and specificity of analog series to select the best candidates for in vivo testing.
Mechanism of Action Studies Investigate whether your compound works by reducing substrate, inhibiting conversion, or enhancing clearance.
Basic Research Explore the fundamental biology of prion strain adaptation, transmission barriers, and cellular propagation pathways.

Features

Features Description
Diverse Strain Portfolio Our cell lines are expertly infected with a wide range of well-documented prion strains, including mouse-adapted scrapie (RML, 22L, ME7), Chronic Wasting Disease (CWD), and Bovine Spongiform Encephalopathy (BSE).
Genetically Engineered for Precision We offer a suite of genetically modified cell lines, including PrP knockout cells engineered to express specific PrP variants from human, ovine, elk, and other species.
High-Permissivity Clones Many of our models are derived from subclones specifically selected for their enhanced ability to support robust prion replication, ensuring consistent and high-level production of the pathological prion protein, PrPSc.
Advanced Human-Relevant Systems Go beyond traditional lines with our cutting-edge human models, including iPSC-derived astrocytes and cerebral organoids, for unparalleled translational relevance.
Rigorous Quality Control Every batch is meticulously validated for the sustained, high-level production of proteinase-K (PK)-resistant PrPSc, with comprehensive characterization data provided to ensure model integrity and performance.
Paired Control Lines Each infected cell model is available with its corresponding uninfected, "cured" parental line, providing the ideal negative control for your experiments.
A picture that presents Microglial activation. (Li, et al., 2021) (OA Literature)Fig.1 Microglial activation in prion disease.1

FAQs

  • Can I use these models to study prion neurotoxicity?
    Most immortalized cell lines are unsuitable for neurotoxicity studies as they do not show significant cytotoxic effects upon prion infection. For this purpose, we strongly recommend our advanced models. Primary neuronal cultures or cerebral organoids possess the complex architecture required to replicate key pathological features like neuronal loss and synaptic dysfunction. These physiologically relevant systems provide a more accurate platform for assessing neurotoxicity, ensuring your results are robust and translatable.
  • How are the cells shipped?
    To ensure maximum viability, our cells are cryopreserved and shipped frozen in cryovials on an abundant amount of dry ice. This maintains a stable, deep-frozen temperature throughout transit. Each shipment includes a comprehensive datasheet with detailed protocols for every critical step, including proper thawing, recommended culture media, and routine maintenance. This provides everything you need for successful cell recovery and experimentation, right out of the box.

Creative Biolabs is dedicated to breaking the logjams in neurodegenerative drug discovery. Our comprehensive portfolio of Prion Disease cell model Products provides an essential platform for generating translationally relevant data, enabling researchers to identify and optimize therapeutic candidates with a higher probability of success. From high-throughput screening in robust mouse models to validating leads against authentic human prions and proactively assessing drug resistance, we provide the critical insights needed to move your project forward with confidence. Contact us today to discuss your project with one of our prion biology specialists and get a personalized quote.

Related Product Sections

For more categories of Neuromuscular & Genetic Disease related Research Tools, please visit the following link:

Reference

  1. Li, Bei, Meiling Chen, and Caihong Zhu. "Neuroinflammation in prion disease." International Journal of Molecular Sciences 22.4 (2021): 2196. DOI: 10.3390/ijms22042196. Use under Open Access license CC BY 4.0, without modification.