Mouse Models
DRPLA
Publication
Transgenic DRPLA 113Q Mice
Identification
- Causal gene(s)
- ATN1
- Repeat size or mutation
- 113Q
- 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
- Propagation of expansion through in vitro fertilization and transplantation creating a subline from the Q76 and Q129 mice
- Promoter: gene
- SV40
Phenotype
- Progression
- progressive
- Hallmark features
- Increased severity of motor deficits and memory disturbance with increasing CAG repeat length and age; reduced survival, reduced body weight, intranuclear inclusions, neuronal dysfunction
Source & Publications
- Originating lab / institution
- The University of Tokyo
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