Why are glial cells like astrocytes and microglia included in your models?

PSP is not just a disease of neurons. Tau aggregates are found in astrocytes and oligodendrocytes, and neuroinflammation is a key factor in disease progression. Furthermore, GWAS have identified risk genes like RUNX2 and MOBP that point directly to microglial and oligodendrocytic dysfunction. Including these cells is crucial for modeling the complete disease environment.

Can I study the Unfolded Protein Response (UPR) with your models?

Yes. The UPR is activated in affected brain regions in PSP, and the gene EIF2AK3 (which encodes the UPR protein PERK) is a confirmed genetic risk locus. Our models provide an excellent system to study how UPR dysfunction contributes to tau accumulation and to test therapies designed to restore its function.

What types of tau does your lab focus on?

Our focus is on the tau species most relevant to PSP: the 4-repeat (4R) isoform and its pathologically N-terminally truncated form.

How is PSP different from other tauopathies like Alzheimer's?

The key difference is the tau isoform involved. PSP is a primary 4R-tauopathy, while Alzheimer's involves both 3R and 4R tau. This genetic and molecular difference likely explains why risk factors, such as the APOE alleles, have opposite effects in the two diseases.

Are your cell models based on primary cells or iPSCs?

We offer a range of models derived from human induced pluripotent stem cells (iPSCs), which can be differentiated into neurons, astrocytes, oligodendrocytes, and microglia. This provides a renewable, scalable, and genetically flexible platform for your research.

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Progressive Supranuclear Palsy (PSP) Cell Model Products

Introduction Types Advantages Applications FAQs Related Product Sections Product List

Introduction

Progressive Supranuclear Palsy (PSP) presents a formidable challenge in neuroscience. As a devastating 4R-tauopathy, it is clinically distinct from Parkinson's disease, yet diagnosis is often delayed by up to three years. With no cure and a lack of effective disease-modifying therapies, there is an urgent unmet need for research tools that can dissect the disease's complex, multifactorial etiology.

At its core, PSP is characterized by the accumulation of misfolded and aggregated 4-repeat (4R) tau in both neurons and glial cells. Driven by a strong genetic underpinning, primarily linked to the MAPT gene, the disease involves a cascade of cellular failures—from disrupted protein clearance to widespread neuroinflammation.

To accelerate the discovery of effective treatments, researchers require disease models that faithfully replicate this multifaceted pathology. Acme NeuroSolutions has engineered a suite of state-of-the-art PSP cell models designed to address the specific pathological pathways implicated by genetics and clinical findings. Move beyond simplistic models and start exploring the true complexity of PSP. Contact our specialists today to discover the perfect PSP model for your research.

Alternatively, specific offerings can be found by directly consulting our comprehensive Product List.

Types of PSP Models

Types	Description
Genetic Background Models	These models incorporate the most significant genetic risk factors identified in human populations. MAPT H1c Model: Carries the H1c sub-haplotype, associated with increased 4R-tau expression. Ideal for general screening and studying tau-centric pathologies. APOE2 Model: Expresses the APOE ε2 allele, which, unlike in Alzheimer's, increases the risk for PSP. Perfect for studying the role of lipid metabolism and glial-neuronal interactions in PSP.
Pathway-Specific Models	These models feature targeted genetic mutations that allow for the precise investigation of cellular systems that fail in PSP. *UPR-Deficient Model (EIF2AK3 KO): A knockout of the EIF2AK3* (PERK) gene, a critical component of the Unfolded Protein Response. Essential for testing therapies aimed at restoring protein homeostasis. *Autophagy-Impaired Model (KANSL1/PLEKHM1 KO)*: Features knockouts of genes critical for the fusion of autophagosomes and lysosomes, directly modeling the impaired cellular clearance central to PSP.
Tau-Variant Models	These models express specific forms of the tau protein implicated in the disease process. Truncated Tau Model: Expresses the N-terminally truncated form of tau found in PSP patient brains, which shows a higher propensity for aggregation. SUMO-Fusion Tau Model: Expresses a tau protein fused to SUMO1, modeling the post-translational modification shown to dramatically increase aggregation when combined with truncation.

Advantages: Accelerate and De-Risk Your PSP Program

Higher Clinical Relevance

By modeling the specific 4R-tau isoform, key genetic risks, and the essential role of glia, our platforms ensure your findings are more predictive of human disease.

Superior Mechanistic Insight

Isolate the impact of specific pathological events, such as the SUMO1-tau interaction or the failure of the ubiquitin-proteasome system via TRIM11 dysfunction, to precisely define your drug's mechanism of action.

Increased Screening Efficiency

Robust, reproducible, and scalable models enable high-throughput screening of compounds designed to target tau aggregation, enhance clearance, or reduce neuroinflammation.

Confident Decision-Making

Generate higher-quality, more reliable data to de-risk your pipeline and make go/no-go decisions with greater confidence before committing to expensive in vivo studies.

By incorporating these advanced features, our models provide significant advantages for therapeutic development.

Applications: Powering Every Stage of Discovery

Applications	Description
High-Throughput & High-Content Screening	Identify novel lead compounds that prevent tau aggregation, block the interaction between truncated tau and SUMO1, or enhance protein clearance.
Mechanism of Action (MoA) Studies	Elucidate the specific cellular consequences of genetic risk factors, providing a deeper understanding of how variants in MAPT, LRRK2, TRIM11, and EIF2AK3 drive pathology.
Biomarker Discovery and Validation	Use conditioned media from our models to identify secreted biomarkers, such as modified tau species, that can address the "scarcity of validated in vivo disease-specific biomarkers" hindering clinical trials.
Preclinical Efficacy Testing	Generate a robust preclinical data package for your lead candidates, including small molecules, antisense oligonucleotides (ASOs), and antibody-based therapies.

A picture that presents the Markers of UPR activation associated with pathological protein deposition in neurodegenerative disorders. (Sidhom, et al., 2022) (OA Literature)

Fig.1 Markers of UPR activation associated with pathological protein deposition in neurodegenerative disorders.¹

FAQs

Why are glial cells like astrocytes and microglia included in your models?
PSP is not just a disease of neurons. Tau aggregates are found in astrocytes and oligodendrocytes, and neuroinflammation is a key factor in disease progression. Furthermore, GWAS have identified risk genes like RUNX2 and MOBP that point directly to microglial and oligodendrocytic dysfunction. Including these cells is crucial for modeling the complete disease environment.
Can I study the Unfolded Protein Response (UPR) with your models?
Yes. The UPR is activated in affected brain regions in PSP, and the gene EIF2AK3 (which encodes the UPR protein PERK) is a confirmed genetic risk locus. Our models provide an excellent system to study how UPR dysfunction contributes to tau accumulation and to test therapies designed to restore its function.
What types of tau does your lab focus on?
Our focus is on the tau species most relevant to PSP: the 4-repeat (4R) isoform and its pathologically N-terminally truncated form.
How is PSP different from other tauopathies like Alzheimer's?
The key difference is the tau isoform involved. PSP is a primary 4R-tauopathy, while Alzheimer's involves both 3R and 4R tau. This genetic and molecular difference likely explains why risk factors, such as the APOE alleles, have opposite effects in the two diseases.
Are your cell models based on primary cells or iPSCs?
We offer a range of models derived from human induced pluripotent stem cells (iPSCs), which can be differentiated into neurons, astrocytes, oligodendrocytes, and microglia. This provides a renewable, scalable, and genetically flexible platform for your research.

Creative Biolabs is dedicated to providing the scientific community with the highest quality, most clinically relevant tools to combat Progressive Supranuclear Palsy. Our genetically engineered cell models, informed by the very latest research, empower you to investigate the core mechanisms of the disease with unparalleled precision and reliability.

Our team of specialists is ready to discuss your specific research needs and help you select the ideal product to advance your project. Contact Our Team for More Information and to Discuss Your Project.