TG-hATXN3 (85Q)
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Age
Genotype
Sex
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Basic Information
Model Name
TG-hATXN3 (85Q)
Product ID
C001397
Gene Name
Background
C57BL/6JCya
Gene Alias
AT3; JOS; MJD; ATX3; MJD1; SCA3
Classification
Other Target Humanized Models,Neurological Disease Models,Rare Disease Models
NCBI ID
Phenotype
Chromosome
14
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Note: When using this mouse strain in a publication, please cite “TG-hATXN3 (85Q) mice (Catalog C001397) were purchased from Cyagen.”
Strain Description
This strain represents a SCA3 disease model generated through transgenic technology, expressing a human ATXN3 gene carrying approximately 85 CAG repeats (Q). The number of CAG repeats (Q) in the human ATXN3 gene in this model is associated with the more severe forms of SCA3. Preliminary research data indicate that 2-month-old TG-hATXN3 (85Q) mice exhibit a significant increase in stride width and a shortened latency to fall in the rotarod test, suggesting impairments in motor coordination and activity. Therefore, this strain can be utilized for research on the ubiquitin-proteasome system and the pathogenic mechanisms of SCA3, as well as for the screening, development, and evaluation of targeted therapeutic drugs.
Application Area
Research on the ubiquitin-proteasome system and the pathological mechanisms of SCA3
Screening, development, and evaluation of therapeutic drugs for SCA3
Validation Data