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TREX1 the major 3' DNA exonuclease in mammalian cells. Normally associates with the endoplasmic reticulum (ER). Translocates to the nucleus at S phase after DNA damage by gamma-irradiation or hydroxyurea. Trex1-deficient cells show defective G1/S transition, with single-stranded DNA molecules persisting after S phase and accumulating in the ER. Degrades ssDNA. Prevents chronic checkpoint activation and inappropriate immune activation. Mutations cause Aicardi-Goutieres syndrome, an autoimmune disorder. Trex1a(-/-) mice have autoinflammatory responses. The gene for this protein is either identical to or adjacent to that of ATRIP. Some of the mRNAs that encode TREX1 also encode ATRIP in another reading frame. Three alternatively spliced human isoforms have been reported. Note: This description may include information from UniProtKB.
Protein type: Cell cycle regulation; DNA repair, damage; DNA-binding; Deoxyribonuclease; EC
Chromosomal Location of Human Ortholog: 3p21.31
Cellular Component: endoplasmic reticulum membrane
Molecular Function: 3'-5' exonuclease activity; 3'-5'-exodeoxyribonuclease activity; metal ion binding; MutLalpha complex binding; MutSalpha complex binding; protein binding; protein homodimerization activity; single-stranded DNA binding
Biological Process: DNA metabolic process; DNA repair; regulation of interferon type I production
Disease: Aicardi-goutieres Syndrome 1; Chilblain Lupus 1; Systemic Lupus Erythematosus; Vasculopathy, Retinal, With Cerebral Leukodystrophy
Reference #:  Q9NSU2 (UniProtKB)
Alt. Names/Synonyms: 3'-5' exonuclease TREX1; DNase III; Three prime repair exonuclease 1; TREX1
Gene Symbols: TREX1
Molecular weight: 38,923 Da
Basal Isoelectric point: 8.78  Predict pI for various phosphorylation states
Protein-Specific Antibodies or siRNAs from Cell Signaling Technology® Total Proteins
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