Mouse Models DRPLA Publication

Atro-118Q transgenic mice

Identification

Causal gene(s)
ATN1
Repeat size or mutation
118Q
Animal model
Mouse

Model Details

Mouse strain / background
C57BL/6J and SJL/J
Type of model
transgenic

Transgenic Construct

Transgenic construct: sequence type
Full length human cDNA
Transgenic construct: details
Full-length human atrophin-1 cDNA with 118Q was cloned into the NSE transgenic vector with a rat neuron-specific enolase promotor
Promoter: gene
NSE
Promoter: species
Rat

Phenotype

Progression
progressive
Hallmark features
Ataxia, tremors, motor defects, nuclear inclusions, somal atrophy, hypoacetylation of histone H3

Source & Publications

Originating lab / institution
Morgan-Tan International Center for Life Sciences

Twenty Twenty-Five

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