Mouse Models DRPLA Publication

AT-FL-65Q

Identification

Research Resource Identifier (RRID)
RRID: MMRRC_000398-MU
Causal gene(s)
ATN1
Repeat size or mutation
65Q
Animal model
Mouse

Model Details

Mouse strain / background
C3H/B6
Type of model
transgenic

Transgenic Construct

Transgenic construct: sequence type
Full-length cDNA
Transgenic construct: details
Full-length cDNA of atrophin-1 encoding 65Q constructs were ligated into the mouse prion protein promoter vector ensuring expression in neurons throughout the brain
Promoter: gene
PrP
Promoter: species
Mouse

Phenotype

Progression
progressive
Hallmark features
Involuntary tremors, ataxic gait, loss of coordination, hyperactivity, increased aggression, lower breeding efficiency, seizures, premature death, atrophin-1 nuclear inclusion bodies

Source & Publications

Originating lab / institution
Johns Hopkins University School of Medicine

Twenty Twenty-Five

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