Huntington Mouse Models

HD models include fragment models (N171-82Q, R6/2 expressing truncated HTT with expanded CAG repeats), full-length knockin models (replacing mouse Htt with human HTT containing expanded CAG), and conditional models (inducible HTT expression). Selection depends on research question, timeline needs, and phenotype severity requirements.

Longer CAG repeats cause earlier and more severe phenotypes. Fragment models with high CAG repeat numbers show rapid progression. Full-length knockin models show slower progression more closely resembling human disease. Age at phenotype onset depends on repeat length and model type. Contact us to discuss the best model for your study timeline.

Yes. HD models enable testing of huntingtin-lowering therapies (ASOs, RNAi, zinc finger repressors), somatic instability modifiers (MSH3 reduction), aggregate clearance strategies, and gene therapy approaches. Models show measurable behavioral, neuropathological, and molecular endpoints for therapeutic evaluation.

Core behavioral tests include rotarod (motor coordination), balance beam (fine motor control), grip strength (forelimb/hindlimb strength), gait analysis (stride length, coordination), open field (activity patterns), and clasping behavior (limb clasping when suspended). Multiple complementary tests strengthen phenotypic characterization. (/request-quote)