Huntington's Disease (HD)

Huntington's disease (HD) is a rare autosomal dominant neurodegenerative disorder primarily affecting the basal ganglia and slowly disrupts motor control and cognitive function. HD predominantly manifests in middle-age and is characterized by a slow but progressive deterioration that ultimately leads to mortality. The disease is triggered by mutations in the huntingtin (HTT) gene situated on chromosome 4, with CAG trinucleotide repeat expansion. GemPharmatech has developed the B6-hHTT130-N model, carrying 130 CAG repeats, to simulate HD symptoms and support therapeutic development.

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Mouse Models Related to Neurological Diseases
200+
Studies Completed
15+
ND-Enabling Studies

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Industry-leading capabilities for Huntington's Disease (HD) disease research

Clinically Relevant PolyQ Model

The B6-hHTT130-N model carries 130 CAG repeats, well above the pathogenic threshold of 40, ensuring robust mutant HTT (mHTT) aggregation and neuronal pathology relevant to human HD.

Comprehensive Behavioral & Pathological Characterization

Our platform provides rotarod, grip strength, and immunohistochemical assessments of mHTT aggregation across multiple brain regions, enabling thorough preclinical efficacy profiling.

Translational Study Design

Our team designs and executes customized protocols — from early presymptomatic to advanced disease stages — to evaluate HTT-lowering therapies, ASOs, and other disease-modifying candidates.

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Case Studies & Validation Data

mHTT Aggregation & Motor Dysfunction Study

Age-Dependent Motor Dysfunction and Mutant HTT Protein Aggregation of B6-hHTT130-N Mice

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Age-Dependent Motor Dysfunction and Mutant HTT Protein Aggregation of B6-hHTT130-N Mice

Panel A & B: Mice exhibited motor dysfunction from 12 weeks of age.

All data represented as mean ± SEM. *P<0.05, **P<0.01, ***P<0.001, ****P<0.0001, two-way ANOVA.

Panel C: Representative brain sections from B6-hHTT130-N mice at different ages by H&E staining. The results showed aggregation of mHTT protein at 8-20 weeks of age. Scale bar: 100 μm.

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