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Creative Biolabs

Transgenic and Non-Human Primate Models in Huntington's Disease Research

Genetic neurodegeneration modeling simulates progressive neurological decline such as Huntington's disease (HD) characterized by motor, cognitive, and psychiatric impairments. These in vivo platforms including transgenic rodent and non-human primate models support molecular studies and preclinical drug evaluation to accelerate translational research.

Creative Biolabs provides you essential animal models which when combined with our advanced in vitro models enable a comprehensive approach to HD research. Explore Reliable Solutions Today!

Transgenic Mouse Models in HD Research

Transgenic mouse models of HD have been used extensively to study HD pathogenesis and to test potential therapies. The most common models differ in terms of their genetic constructs, the phenotypes that they develop and their relevance to human HD.

Table 1 Key transgenic mouse models

Model Genetic Modification Phenotypic Features Advantages Limitations
R6/2 Human HTT exon 1 fragment with ~150 CAG repeats Early onset motor deficits, cognitive decline, seizures, weight loss, inclusion bodies, death ~15 weeks. Rapid disease progression; model of juvenile HD. Robust phenotype, widely studied, fast progression. Robust phenotype, widely studied, fast progression.
N171-82Q N-terminal 171 amino acid fragment of human mHTT with 82 CAG repeats Tremors, hypokinesia, lack of coordination, striatal atrophy, no seizures, failure to gain weight. Larger fragment than R6/2, better striatal degeneration representation. No seizures; disease progression slower than R6/2.
YAC128 Full-length human HTT with 72–128 CAG repeats Progressive motor and cognitive deficits starting 2–3 months, striatal and cortical atrophy, selective MSN loss. Expresses full-length mutant HTT with regulatory elements, better mimics human disease progression. Moderate disease progression; maintenance can be complex.
BACHD Full-length human HTT with 97 CAG/CAA repeats Progressive motor deficits, psychiatric-like symptoms, cortical and striatal atrophy, weight gain (unique). Full-length mutant HTT with slower progression; models psychiatric symptoms. Unique weight gain phenotype may differ from human disease.
Knock-in (KI) Mouse HTT gene with expanded CAG repeats (140–175) Motor abnormalities, hyperactivity, gait abnormalities, neuronal loss by 2 years, synaptic deficits. Genetically precise, mimics endogenous expression. Slower disease progression, longer study duration required.

Figure 1 Investigation of astrocyte contributions to Huntington's disease pathology in the striatum of R6/2 mouse model showing altered astrocyte function (OA Literature)Figure 1 Investigation of astrocyte contributions to Huntington's Disease pathology using the R6/2 mouse model.1,3

To support research using such models, Creative Biolabs offers a comprehensive portfolio of Huntington's disease-related antibodies designed for precise molecular and cellular analyses in transgenic mouse tissues. These reagents facilitate mechanism of action (MOA) studies and neuroinflammation assays aimed at analyzing glial activation and inflammatory pathways. By empowering researchers to probe pathogenic mechanisms and validate therapeutic targets across diverse HD models, our antibodies accelerate discovery and therapeutic development. Contact us today to learn how our custom solutions can support your HD research and drug development goals.

Cat. No Product Name Host Application
NAB2105185SL Mouse Anti-Human CREBBP Monoclonal Antibody (CBP5695) Monoclonal DB; ICC; IP; WB
NAB2105186SL NeuroMab™ Mouse Anti-CREBBP (CBP5696) Monoclonal IF; IHC
NAB2105187SL NeuroMab™ Mouse Anti-Human CREBBP (CBP5697) Monoclonal ELISA; IF; WB
NAB2105189SL Mouse Anti-Human CREBBP Monoclonal Antibody (CBP5698) Monoclonal ICC; IP; WB
NRP-0322-P1903 Anti-CREBBP Monoclonal Antibody (CBP8020) Monoclonal WB; IHC; ICC; IP
NAB2007FY665 Mouse Anti-VDAC1 Monoclonal Antibody (CBP2022) Monoclonal WB; IHC; ICC; IF
NAB2010354LS NeuroMab™ Mouse Anti-VDAC1 Monoclonal Antibody (CBP2356) Monoclonal WB; IHC-P
NRP-0422-P458 NeuroMab™ Anti-VDAC1, Clone N152B/23 (CBP8559) Monoclonal WB; IHC; ICC
NRZP-0922-ZP4706 NeuroMab™ Anti-VDAC Antibody, Clone N29361 (CBP16017) Monoclonal WB; IHC
NRZP-0423-ZP458 NeuroMab™ Anti-VDAC1 BBB Shuttle Antibody, Clone N152B/23 Monoclonal WB; IHC; ICC
NAB2007FY1703 Mouse Anti-REST Monoclonal Antibody (CBP6754) Monoclonal IHC; IF
NAB20101997CR Mouse Anti-HIP1 Monoclonal Antibody (CBP2919) Monoclonal IHC; WB; ELISA
NAB-2103-P365 NeuroMab™ Mouse Anti-HIP1 Monoclonal Antibody (CBP4770) Monoclonal WB; IP; IF; IHC-P
NAB-2103-P370 NeuroMab™ Mouse Anti-HIP12 Monoclonal Antibody (CBP4775) Monoclonal WB; IP
NAB-0421-R0639 NeuroMab™ Mouse Anti-HIP7 Monoclonal Antibody (CBP6056) Monoclonal IHC; WB

Transgenic Rat Models in HD Research

While mouse models have been the workhorse of HD research, transgenic rat models offer valuable complementary systems due to their larger brain size and more complex behavior, which can facilitate certain neurophysiological and imaging studies.

BACHD Transgenic Rat Model

The BACHD rat model is also a transgenic animal that is full-length human mutant HTT gene with 97 CAG/CAA repeats along with its regulatory elements. BACHD rats develop early signs of HD disease with motor impairments and anxiety-like behaviors and form mutant huntingtin protein aggregates in cortical and striatal regions.

Advantages of Transgenic Rat Models

  • Larger brain size
  • Slower and often adult-onset disease progression
  • More complex behavior
  • Effective for longitudinal studies and therapeutic interventions like neurotransplantation

Non-Human Primate Models in HD Research

Non-human primate (NHP) models of HD represent a significant advancement in modeling HD due to their close genetic, physiological, and neurological similarity to humans, which rodent models cannot fully replicate.

Figure 2 Overview of advancements in neurodegenerative disease non-human primate models enabled by optimized transgenesis and somatic cell nuclear transfer techniques (OA Literature)Figure 2 Advancing neurodegenerative disease NHP models through optimized transgenesis and nuclear transfer techniques.2,3

Rationale for Using NHP Models in HD Research

  • Closer genetic, anatomical, and physiological similarity to humans compared to rodents, especially in brain structure and circuitry affected in HD (striatum, cortex, basal ganglia).
  • Ability to model complex motor, cognitive, and psychiatric symptoms analogous to human HD, including chorea, dystonia, working memory decline, and neuropsychiatric disturbances.
  • Longer lifespan and larger brain size allow for longitudinal studies and advanced neuroimaging.
  • Provide a higher translational value for testing therapies and biomarkers before clinical trials.

Genetic Engineering Approaches

Genetic engineering techniques have become essential tools to develop NHP models for HD. Each approach offers unique advantages and faces specific limitations, which must be carefully considered in experimental design.

Table 2 Genetic engineering methods and their pros and cons in NHP Huntington's disease models

Approach Description Advantages Limitations
Transgenic Monkey Integration of mutant human HTT gene with expanded CAG repeats into rhesus macaques via lentiviral vectors.
  • Stable expression of mutant huntingtin
  • Models progressive HD pathology and symptoms
  • Technical challenges
  • Low efficiency
  • Long generation time
  • Ethical concerns
Viral Vector-Mediated Models Injection of recombinant adeno-associated viral vectors (AAV2, AAV2.retro) expressing mutant huntingtin fragments into adult macaque striatum.
  • Rapid induction of mutant huntingtin expression
  • Targeted brain regions
  • Flexible experimental design
  • Limited germline transmission
  • Focal pathology
  • Ethical considerations

Recent Advances and Models in HD Research

Creative Biolabs continuously integrates cutting-edge methodologies to develop and utilize advanced HD models. These models enable detailed investigation of disease progression and pathology, supporting translational research efforts to identify effective therapies.

Table 3 Recent advance and models in HD research

Model/Study Methodology Key Findings
AAV2/AAV2.retro mHTT macaque model Stereotactic injection of AAV vectors expressing mutant HTT fragments into caudate and putamen. Progressive motor and cognitive decline over 20–30 months; widespread mHTT aggregates; white and gray matter degeneration.
Transgenic rhesus macaque model Lentiviral integration of full-length mutant HTT with expanded CAG repeats. Expression of hallmark HD pathology including nuclear inclusions; motor symptoms like chorea and dystonia; cognitive decline.
Striatal interneuron studies Histological analysis of HD monkeys' striata. Altered interneuron populations correlating with neuronal loss, similar to human HD pathology.

NHP Huntington's disease models offer unparalleled biological relevance due to their close genetic and physiological similarity to humans, making them invaluable tools for mechanistic studies and therapeutic testing. At Creative Biolabs, we provide customized NHP HD model services designed to help you leverage these strengths while addressing inherent challenges in such complex systems.

We also specialize in mHTT aggregation lowering and huntingtin splicing analyses to support cutting-edge therapeutic strategies. Our expertise extends to using HD-related cell models integrated with in vivo rodent and large animal models, providing a comprehensive platform for understanding disease progression and evaluating treatment responses across multiple biological systems.

Cat. No Product Name
NRYF-0324-HZ9 Human mHTT-92Q (exon 1) Stable Cell Line
NRZP-0323-ZP7 iNeuTM Glutamatergic Neurons HTT 50CAG/WT, HTT Model
NRZP-0622-ZP77 iNeuTM Human Neural Stem Cells - Huntington's Disease Patient, Donor 50

Comparative Analysis of Transgenic Rodent and Non-Human Primate Models

Selecting the appropriate animal model is crucial to the success of neurodegenerative disease research. Creative Biolabs offers comprehensive services encompassing transgenic rodent and NHP models, providing expert support to help you choose and utilize the optimal system tailored to your specific research goals.

Table 4 Comparison of transgenic rodent and NHP models

Model Type Advantages Limitations
Transgenic Mice Genetic tools, cost-effective, rapid breeding Limited behavioral complexity, short lifespan
Transgenic Rats Larger brain, complex behaviors Fewer models, higher costs
Non-Human Primates High translational relevance, complex cognition Ethical issues, cost, long generation time

Applications of Animal Models in Huntington's Disease Research

Our comprehensive range of rodent and large animal models provide powerful platforms for unraveling disease mechanisms, evaluating therapeutic candidates, and identifying relevant biomarkers. Leveraging these models, we support your research from early discovery through preclinical validation to accelerate translational success.

Table 5 Applications of animal models in different aspects

Application Area Rodent Models Large Animal Models (Pigs, NHPs)
Mechanistic studies Rapid genetic manipulation, molecular pathways Closer neuroanatomy, long-term disease modeling
Therapeutic testing High-throughput screening, gene therapy proof-of-concept Better CNS drug delivery modeling, safety studies
Biomarker development Molecular and behavioral markers Imaging and physiological biomarkers
Behavioral phenotyping Motor and cognitive assays Complex motor and cognitive behaviors
Translational validation Initial efficacy and toxicity Preclinical confirmation before human trials

In conclusion, animal models effectively recapitulate key aspects of Huntington's disease, enabling in-depth mechanistic studies and accelerating therapeutic discovery. Whether working with rodent models for genetic research or large animal models for translational applications, having the right expertise and resources is essential. At Creative Biolabs, we offer comprehensive, end-to-end support to advance your HD research projects, integrating advanced animal models with complementary cellular systems to provide a robust and holistic platform for understanding disease progression and evaluating novel treatments.

Partner with us to leverage state-of-the-art tools and personalized solutions that facilitate your translational success from discovery to preclinical validation.

References

  1. Skotte, Niels H., et al. "Integrative Characterization of the R6/2 Mouse Model of Huntington's Disease Reveals Dysfunctional Astrocyte Metabolism." Cell Reports, vol. 23, no. 7, May 2018, pp. 2211–24. https://doi.org/10.1016/j.celrep.2018.04.052.
  2. Li, Bang, et al. "Modeling Neurodegenerative Diseases Using Non-Human Primates: Advances and Challenges." Ageing and Neurodegenerative Diseases, vol. 2, no. 3, 2022, p. 12. https://doi.org/10.20517/and.2022.14.
  3. Distributed under Open Access license CC BY 4.0, without modification.

Created July 2025

For Research Use Only. Not For Clinical Use.
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