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R6/2 Transgenic Mouse Model of HD
The R6/2 Transgenic Mouse Model is one of the most widely used and well-characterized models for studying Huntington’s disease (HD). This model carries a truncated form of the mutant huntingtin (HTT) gene with an expanded CAG repeat, which is responsible for the neurodegenerative processes seen in Huntington’s disease. The R6/2 mouse exhibits many of the hallmark features of HD, including progressive motor dysfunction, cognitive decline, and neurodegeneration.
Key Features of the R6/2 Model:
- Early and Progressive Onset: Mice typically begin showing symptoms as early as 5–6 weeks of age, including motor coordination deficits, weight loss, and cognitive impairments. Symptoms progressively worsen, providing a window for testing potential therapies.
- Neuropathology: The model replicates significant neurodegeneration in regions like the striatum and cortex, which are heavily affected in human HD. It also exhibits protein aggregates and neuronal inclusions that are characteristic of HD pathology.
- Behavioral Deficits: R6/2 mice display progressive motor dysfunction, including tremors, abnormal gait, and coordination issues, making them ideal for studying motor impairments. Cognitive and psychiatric symptoms, such as anxiety and depression-like behavior, are also observed.
- Therapeutic Relevance: The R6/2 model is frequently used for preclinical testing of potential HD therapies, including gene-targeting approaches, small molecules, and neuroprotective agents. Its rapid disease progression allows for quicker therapeutic evaluation.
This model continues to be instrumental in advancing our understanding of Huntington's disease and developing new treatments. Naason Science has completed validation of R6/2 mouse line for the study of HD; the model paradigm has a complete functional and behavioral battery, imaging (MRI/MRS) and histological and molecular analysis of various regions of brain.
Naason Science model assays:
- Grip Strength
- RotaRod
- Home cage rearing
- Home cage locomotor activity
- Imaging (MRI/MRS, PET-SPECT / CT)
- Histology / Stereology
Study design
TG (zQ175) HD Model
The TG (zQ175) Mouse Model of Huntington’s Disease is a widely utilized transgenic model that carries the full-length human mutant huntingtin (HTT) gene with an expanded CAG repeat, closely mimicking the genetic mutation found in HD patients. The zQ175 model represents a slower progression of the disease compared to other models, making it an excellent tool for studying long-term therapeutic interventions and disease mechanisms.
Key Features of the zQ175 Model:
- Genetic Accuracy: The zQ175 model incorporates the full-length mutant HTT gene, providing a more genetically accurate representation of Huntington’s disease compared to truncated models like R6/2.
- Progressive Onset: This model develops HD symptoms more slowly, starting with subtle motor and cognitive deficits that gradually worsen with age, allowing for the study of disease progression over an extended period.
- Neuropathology: Similar to human HD, zQ175 mice exhibit neuronal degeneration in the striatum and cortex, along with the formation of mutant huntingtin aggregates. The model also shows signs of neuroinflammation, which is an emerging area of interest in HD research.
- Behavioral Deficits: zQ175 mice display progressive motor dysfunction, including impaired coordination, abnormal gait, and reduced grip strength. Cognitive impairments and psychiatric-like symptoms, such as anxiety and depression, are also observed, making this model useful for studying both motor and non-motor aspects of HD.
- Therapeutic Relevance: Due to its slower progression, the zQ175 model is ideal for evaluating long-term therapeutic strategies, including gene-editing techniques, small molecule therapies, and neuroprotective agents. It is particularly valuable for testing treatments that aim to slow or halt disease progression.
This model is crucial for advancing our understanding of the molecular mechanisms of Huntington’s disease and for evaluating promising therapeutic interventions in a setting that closely mirrors human HD pathology.