EXPERIMENTAL MODELS

Contributor Information
- Name Tomas Lindahl
- Institute Cancer Research UK, London Research Institute: Clare Hall Laboratories
Tool Details
- Tool name: Trex1 -/- Mouse
- Tool type: Experimental models
- Tool sub-type: Mouse
- Disease: Antiviral-like autoimmunity; Autoinflammatory disease; Aicardi-Goutieres syndrome
- Model: Knock-Out
- Conditional: No
- Genetic background and cross history: Standard gene-targeting in ES cells using a genomic DNA construct flanking the Trex1 locus in which amino acids 103 to 207 of the Trex1 ORF were replaced by a neomycin resistance gene/polyA signal.
- Phenotype: Autoimmunity manifested as inflammatory myocarditis / reduced lifespan
- Zygosity: Homozygous
- Description: Model for the human recessive autoimmune disease Aicardi-Goutieres syndrome; potential use for testing of therapeutic agents; identifies a novel immune pathway. Trex1 KO mouse
- Research area: Drug development; Genetics; Immunology
- Production details: Standard gene-targeting in ES cells using a genomic DNA construct flanking the Trex1 locus in which amino acids 103 to 207 of the Trex1 ORF were replaced by a neomycin resistance gene/polyA signal.
- For Research Use Only
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References
- • Yang et al. 2007. Cell. 131(5):873-86. PMID: 18045533.
- • Trex1 exonuclease degrades ssDNA to prevent chronic checkpoint activation and autoimmune disease.
- • Morita et al. 2004. Mol Cell Biol. 24(15):6719-27. PMID: 15254239.
- • Gene-targeted mice lacking the Trex1 (DNase III) 3'-->5' DNA exonuclease develop inflammatory myocarditis.