What CAG repeat lengths are available in your HD models?

We offer models with a variety of CAG repeat lengths, including those in the reduced penetrance range (e.g., 37 CAGs) and fully penetrant range (e.g., 51, 72 CAGs). Please inquire about specific repeat lengths.

Do these models exhibit known HD pathologies?

Yes, our models are characterized to display key HD pathologies such as the expression of mutant huntingtin protein (mHTT), mHTT aggregation, and somatic CAG repeat instability in differentiated neural cells.

Can Creative Biolabs develop custom HD cell models?

Yes, we offer custom model development services, including generating iPSC lines from specific patient cohorts or engineering specific genetic modifications.

Can these models be used to study early HD pathogenesis?

Yes, iPSC and hESC-derived models are exceptionally well-suited for studying the very early stages of HD pathogenesis at a cellular and molecular level, aligning with the biomarker-defined stages of the HD-ISS framework.

What are the main advantages of using iPSC-derived HD models?

Key advantages include their patient-specificity, ability to carry the endogenous HD mutation, differentiation potential into multiple relevant cell types, and their power in drug screening and creating isogenic controls.

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Huntington's Disease (HD) Cell Model Products

Introduction Types Acquirement Advantages Applications FAQs Related Product Sections Product List

Introduction

Huntington's disease (HD) is a devastating, inherited neurodegenerative disorder characterized by progressive motor dysfunction, cognitive decline, and psychiatric disturbances. Caused by an expansion of a CAG (cytosine-adenine-guanine) trinucleotide repeat in exon 1 of the huntingtin gene (HTT), HD follows an autosomal dominant inheritance pattern, meaning that children of an affected parent have a 50% chance of inheriting the mutated gene. The disease typically manifests in mid-life, between 30 and 50 years of age, and relentlessly progresses, with death often occurring 10-20 years after clinical onset. Currently, there are no cures for HD, and available treatments only manage symptoms rather than altering the disease course.

At Creative Biolabs, we are dedicated to empowering the scientific community in the fight against Huntington's Disease. We offer a comprehensive portfolio of cutting-edge HD cell models, meticulously developed to help researchers explore disease pathogenesis, identify novel therapeutic targets, and accelerate the journey towards effective treatments. Discover our tailored cell model solutions today.

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

Types of HD Cell Models

Types	Description
Immortalized Human Cell Lines (e.g., H4, HEK293)	Patient-Derived Cells: We offer well-characterized lymphoblasts derived from HD patients, which are valuable tools for studying systemic defects like mitochondrial abnormalities and increased apoptotic susceptibility.
Genetically Engineered Cell Lines	Our portfolio includes various cell lines engineered to express mutant huntingtin, as well as striatal progenitor models ideal for investigating key disease mechanisms like mitochondrial fragmentation and cristae derangement.
Human Pluripotent Stem Cell (hPSC)-Derived Models	Access our HD human embryonic stem cells (hESCs), derived from embryos screened by preimplantation genetic diagnosis (PGD). These powerful lines differentiate into affected neural cell types and enable the study of early pathological events like CAG repeat instability in neural progenitors.
Specialized Models for Advanced Studies	We provide models specifically designed to investigate mitochondrial dysfunction pathways and to support research aligned with novel frameworks like the HD Integrated Staging System (HD-ISS) for studying pre-clinical disease stages.

Acquiring Your HD Cell Models

Advantages of Partnering with Creative Biolabs for HD Cell Models

Enhanced Human Relevance

Move beyond the limitations of animal models by studying HD in a true human cellular context, increasing the translational potential of your findings.

Accelerated Discovery Timelines

Our ready-to-use, well-characterized models enable faster initiation of experiments, facilitating rapid screening of therapeutic compounds and deeper mechanistic insights.

Insight into Early Disease Mechanisms

Utilize our models to investigate HD's earliest molecular changes before symptom onset, aligning with modern, biomarker-defined staging systems like the HD-ISS.

Support for Personalized Medicine

Patient-derived iPSC models allow for the exploration of disease variability and differential drug responses based on individual genetic backgrounds.

Cost-Effective Research Solutions

Save time and money with our in vitro models. They reduce reliance on expensive animal studies, especially for high-throughput screening and validation.

Platform for Next-Generation Therapies

Our models provide a platform for developing and validating next-generation therapies, including gene editing (ASOs, CRISPR/Cas9) and cell-based regenerative approaches.

Reliable and Reproducible Results

Benefit from standardized, quality-controlled cell models that ensure consistency and reproducibility in your experiments.

Choosing Creative Biolabs' HD cell models offers distinct advantages for your research:

Applications: Driving HD Research Forward

Creative Biolabs' HD cell models are versatile tools for a wide array of research applications:

Applications	Description
Fundamental Disease Modeling	Investigating the molecular and cellular underpinnings of HD, including mHTT toxicity, protein aggregation, transcriptional dysregulation, mitochondrial dysfunction, impaired proteostasis, and CAG repeat dynamics. Studying the selective vulnerability of specific neuronal populations, particularly medium spiny neurons.
Drug Discovery and Development	High-throughput screening (HTS) and high-content screening (HCS) to identify novel therapeutic compounds that can modify disease phenotypes (e.g., reduce mHTT levels, prevent aggregation, improve cell survival). Validating potential drug targets and elucidating mechanisms of action for candidate drugs.
Therapeutic Strategy Development	Developing and testing gene therapy approaches, such as those utilizing ASOs, shRNA, or CRISPR/Cas9 to silence or correct the mutant HTT gene. Evaluating the potential of cell-based therapies, including the transplantation of genetically corrected iPSC-derived neural progenitors or healthy NSCs.
Biomarker Research	Identifying and validating molecular or cellular biomarkers for early diagnosis, tracking disease progression, or measuring therapeutic response in a human system.
Neurodevelopmental Studies	Using iPSC-derived neural cells and brain organoids to explore potential neurodevelopmental abnormalities conferred by the mHTT gene from the earliest stages.
Neurotoxicity and Protective Factor Screening	Assessing the neurotoxic effects of various agents on HD-affected human cells. Screening for neuroprotective compounds or factors that enhance neuronal survival and function.

A picture that presents the Transplantation of genetically corrected Induced pluripotent stem cells. (Csobonyeiova, et al., 2020) (OA Literature)

Fig.1 Transplantation of genetically corrected Induced pluripotent stem cells (iPSCs)– neural progenitor cells (NPCs)/neural stem cells (NSCs) into the affected lesion of the brain.¹

FAQs

What CAG repeat lengths are available in your HD models?
We offer models with a variety of CAG repeat lengths, including those in the reduced penetrance range (e.g., 37 CAGs) and fully penetrant range (e.g., 51, 72 CAGs). Please inquire about specific repeat lengths.
Do these models exhibit known HD pathologies?
Yes, our models are characterized to display key HD pathologies such as the expression of mutant huntingtin protein (mHTT), mHTT aggregation, and somatic CAG repeat instability in differentiated neural cells.
Can Creative Biolabs develop custom HD cell models?
Yes, we offer custom model development services, including generating iPSC lines from specific patient cohorts or engineering specific genetic modifications.
Can these models be used to study early HD pathogenesis?
Yes, iPSC and hESC-derived models are exceptionally well-suited for studying the very early stages of HD pathogenesis at a cellular and molecular level, aligning with the biomarker-defined stages of the HD-ISS framework.
What are the main advantages of using iPSC-derived HD models?
Key advantages include their patient-specificity, ability to carry the endogenous HD mutation, differentiation potential into multiple relevant cell types, and their power in drug screening and creating isogenic controls.

The path to understanding and treating Huntington's Disease is complex, requiring innovative tools and approaches. Creative Biolabs is committed to providing the scientific community with high-quality, well-characterized, and clinically relevant HD cell models. From patient-derived iPSCs and their differentiated neural progeny to advanced 3D organoid systems and genetically engineered lines, our portfolio is designed to accelerate your research from basic mechanistic studies to preclinical drug development. Contact our scientific team today to discuss your specific research needs, explore our customizable solutions, and discover how our advanced Huntington's Disease cell models can empower your next breakthrough.