Huntington's Disease Model
Huntington's disease is a slow-onset genetic neurodegenerative disorder. Since the pathological mechanisms of the disease are still unclear, there is a lack of effective and fundamental treatments to halt the pathological process of Huntington's disease. Therefore, the establishment of animal models that accurately mimic disease phenotypes is essential for exploring the pathological mechanisms of disease and developing effective treatments.
Our company' scientific team leverages our extensive knowledge and specialized evaluation methods to provide clients with reasonable and reliable animal models of Huntington's disease to facilitate their research in related diseases.
Introduction of Huntington's Disease
Huntington's disease is an autosomal dominant neurodegenerative disorder. The main pathological mechanism is an abnormal expansion and unstable CAG repeat sequence within the coding region of the gene encoding Huntington's protein on chromosome 4 of the patient, which affects the normal function of nerve cells leading to the development of HD. The main features of Huntington's disease involve neurological lesions in the striatum and cortex as well as other brain regions, and are accompanied by a decline in cognitive, motor, and neurological functions. The prevalence in the population is about 1 in 10,000.
Fig. 1 Mouse models of Huntington disease. (Ehrnhoefer, 2009)
The Huntington's Disease Models We Provide
Animal models of Huntington's disease provide new insights into Huntington's disease pathology and treatment strategies. Among them, rodent transgenic models are the most popular models of HD. The mouse models of HD are mainly divided into two categories.
Our company is committed to applying a variety of methods to create the most appropriate animal models for Huntington's disease to help clients obtain meaningful results and provide recommendations from model researches.
The following table describes the mouse models of Huntington's disease that we offered.
| Mouse Models | Molecular Mutation |
|---|---|
| R6/2 HD mouse model | Mutant human huntingtin fragment containing 1 kb of 5' UTR region and a polyglutamate-repeat (CAG) expansion of 160 +/- 5, and 262 bp of intron 1. |
| R6/2 transgenic HD mouse model | Expresses mutant human huntingtin (HTT) gene containing a polyglutamate-repeat (CAG) expansion of 120 +/- 5. |
| R6/1 transgenic HD mouse model | Expresses mutant human huntingtin (HTT) gene containing a polyglutamate-repeat (CAG) expansion of 115-150 in the 5' end of the gene. |
| HD-N171-82Q-81 transgenic mouse model | Expresses N-terminally truncated (first 171 amino acids) human huntingtin (HTT) cDNA encoding 82 glutamines. |
| YAC128 transgenic mouse model | Expresses a yeast artificial chromosome (YAC) containing a full-length human huntingtin gene modified with a 128 CAG repeat expansion under the control of the endogenous human HDH promoter. |
Evaluation Methods for The Huntington's Disease Models
Behavioral methods provide a good assessment tool for evaluating animal models of Huntington's disease and can be used to assess the degree of similarity to human disease. Our company uses a variety of advanced experimental equipment and methods for detecting the behavior of Huntington's disease models. We offer behavioral assessment methods including, but not limited to the following.
| Behavior | Testing Methods | ||
|---|---|---|---|
| Cognitive | Morris water maze test | Barnes maze test | Radial arm maze test |
| Y-maze test | T-maze test | 5-CSRTT test | |
| Touch screen test | |||
| Anxiety | Elevated plus maze test | Open field test | Light-dark box test |
| Sucrose preference test | Forced swimming test | Tail suspension test | |
| Social | Three-chamber test | Social interactions test | |
| Motor | Beam test | Rotarod test | General locomotor test |
| Gait analysis test | Pole test | Rearing cylinder test | |
| Ladder rung walking test | |||
Our company is committed to combining our specialized experimental platform and extensive scientific knowledge to jointly provide effective research models for the development of the field of neuroscience. If you are interested, please contact us and describe your specific research, and we will be happy to assist you.
Reference
- Ehrnhoefer, D. E.; et al. Mouse models of Huntington disease: variations on a theme. Disease models & mechanisms. 2009, 2(3-4): 123-129.