Mouse Models
DRPLA
Publication
Transgenic DRPLA 76Q Mice
Identification
- Causal gene(s)
- ATN1
- Repeat size or mutation
- Q76
- Animal model
- Mouse
Model Details
- Mouse strain / background
- C57BL/6J
- Type of model
- transgenic
Transgenic Construct
- Transgenic construct: sequence type
- Entire mutant DRPLA gene
- Transgenic construct: details
- A clone containing the entire mutant DRPLA gene with 78 repeats, a SV40 promoter and neomycin gene
- Promoter: gene
- SV40
Phenotype
- Hallmark features
- Intergenerational and somatic instabilites of expanded CAG repeats
Source & Publications
- Originating lab / institution
- Niigata University
- Links to publications or public resources
-
- Transgenic mice harboring a full-length human mutant DRPLA gene exhibit age-dependent intergenerational and somatic instabilities of CAG repeats comparable with those in DRPLA patients - PubMed
- Severe neurological phenotypes of Q129 DRPLA transgenic mice serendipitously created by en masse expansion of CAG repeats in Q76 DRPLA mice - PubMed
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