Mouse Models FXTAS Publication

Gfa2-CGG99-eGFP mice

Identification

Causal gene(s)
FMR1
Repeat size or mutation
Human expanded CGG99 trinucleotide repeat segment
Animal model
Mouse

Model Details

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

Transgenic Construct

Transgenic construct: sequence type
human expanded CGG99 trinucleotide repeat segment specifically in the astrocytes and Bergmann glia
Transgenic construct: details
Construct containing human expanded (CGG)99 and a GFP sequence under the Gfa2 promoter ensuring restricted expression in astrocytes and Bergmann glia
Promoter: gene
Gfa2
Promoter: species
Human

Phenotype

Hallmark features
Impaired motor performance on ladder-rung test, improved motor performance on rotarod, intranuclear inclusions

Source & Publications

Originating lab / institution
University of California

Twenty Twenty-Five

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