Mouse Models
FXTAS
Publication
L7CGG90Fmr1
Identification
- Causal gene(s)
- FMR1
- Repeat size or mutation
- Specific expression of CGG repeats in Fmr1 in Purkinje neurons
- Animal model
- Mouse
Model Details
- Mouse strain / background
- C57BL/6J
- Type of model
- transgenic
Transgenic Construct
- Transgenic construct: sequence type
- human (CGG)98 repeat
- Transgenic construct: details
- FLAG-tagged Fmr1 with (CGG)90 repeats cDNA constructs under the L7/pcp2 promoter
- Promoter: gene
- L7/pcp2
- Promoter: species
- Mouse
Phenotype
- Progression
- progressive
- Hallmark features
- Intranuclear inclusions in Purkinje neurons, purkinje cell death, behavioral deficits
Source & Publications
- Originating lab / institution
- Baylor College of Medicine
- Links to publications or public resources
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