Mouse Models
DRPLA
Publication
AT-FL-65Q;GFP-LC3
Identification
- Causal gene(s)
- ATN1
- Repeat size or mutation
- 65Q with GFP-LC3
- Animal model
- Mouse
Model Details
- Type of model
- transgenic
Transgenic Construct
- Transgenic construct: sequence type
- Full-length cDNA for Atn1 and DNA fragment for GFP-LC3
- 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; A 1.8-kbp DNA fragment containing the rat LC3 cDNA fused to EGFP at the N-terminus (GFP-LC3)
Phenotype
- Hallmark features
- Stalled canonical autophagy shown by accumulation of p62, downregulation of LC3-I/II conversion and reduced Tfeb expression
Source & Publications
- Originating lab / institution
- King's College London
- Links to publications or public resources
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