Mouse Models
DRPLA
Publication
Transgenic DRPLA 129Q Mice
Identification
- Causal gene(s)
- ATN1
- Repeat size or mutation
- 129Q
- 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
- Accidental expansion of 76Q repeat at the early embryonic stage resulted in a Q129 mouse
- Promoter: gene
- SV40
Phenotype
- Progression
- progressive
- Hallmark features
- myoclonic movements, ataxia, epilepsy, dysfunction of pallidal and cerebellar neurons, dysfunction of synaptic transmission in hippocampal CA1 pyramidal cells, intranuclear inclusions
Source & Publications
- Originating lab / institution
- Niigata University
- Links to publications or public resources
Some fields are restricted to approved researchers. Log in to view them.