Affinage

TREX1

Three-prime repair exonuclease 1 · UniProt Q9NSU2

Length
314 aa
Mass
33.2 kDa
Annotated
2026-06-10
100 papers in source corpus 44 papers cited in narrative 44 extracted findings
Cross-family judge vs UniProt: Affinage preferred faithfulness: 8/8 claims corpus-supported (100%)

Mechanistic narrative

Synthesis pass · prose summary of the discoveries below

TREX1 is the major mammalian 3'→5' DNA exonuclease, a homodimer with a DEDDh-family active site that constitutively degrades cytosolic DNA to prevent cell-autonomous innate immune activation (PMID:10391904, PMID:11279105, PMID:16845398). Biochemically it excises 3'-terminal nucleotides from single-stranded DNA, nicked and blunt dsDNA, and reverse-transcribed DNA, processively melting and digesting duplex substrates to completion via residues including Arg128, Arg174/Lys175, and the Leu24-Pro25-Ser26 cap (PMID:18805785, PMID:21808053, PMID:29734329); crystal structures of substrate/product complexes establish the molecular basis for 3'-nucleotide specificity and DNA strand separation (PMID:17293595, PMID:25848017, PMID:35879334). The dimer interface is functionally integrated, with Arg62 and Arg114 acting in trans across protomers, which explains how dominant-negative disease heterodimers inactivate wild-type enzyme (PMID:21937424, PMID:24616097). Beyond DNA, TREX1 is also an exoribonuclease degrading ssRNA and DNA/RNA hybrids but not dsRNA, with RNA's 2'-OH-driven rigidity reducing catalytic efficiency (PMID:25855793, PMID:37870446). The enzyme functions as an essential negative regulator of the interferon-stimulatory DNA response: loss of TREX1 causes accumulation of endogenous retroelement-derived, micronuclear, and mitochondrial DNA that is sensed by cGAS, driving STING/TBK1/IRF3-dependent type I IFN and lethal autoimmunity, with cGAS deletion fully rescuing the Trex1⁻/⁻ phenotype (PMID:18724932, PMID:24813208, PMID:26223655, PMID:37652009). TREX1 is normally ER-anchored through a C-terminal transmembrane domain; this tethering directs it to ruptured micronuclei and nuclear envelope rupture sites to resect exposed DNA and limit cGAS access, and disease mutations that untether the enzyme abolish this localization (PMID:33476576, PMID:34551315). A separate, nuclease-independent C-terminal interaction with the ER oligosaccharyltransferase (OST) complex — regulated by mitotic CDK1 phosphorylation at Ser261 — stabilizes OST and suppresses glycan-driven immune activation (PMID:26320659, PMID:28297665). TREX1 loss-of-function or C-terminal frameshift mutations cause Aicardi-Goutieres syndrome, familial chilblain lupus, systemic lupus-like disease, and retinal vasculopathy with cerebral leukodystrophy (RVCL), the latter through nuclear mislocalization that disrupts homology-directed repair (PMID:16845398, PMID:25848017, PMID:26320659, PMID:38824133).

Mechanistic history

Synthesis pass · year-by-year structured walk · 22 steps
  1. 1999 High

    Established that TREX1 is an autonomous 3'→5' DNA exonuclease, defining the molecular activity that anchors all later biology.

    Evidence Recombinant protein from E. coli with in vitro exonuclease assays on multiple substrates

    PMID:10391904

    Open questions at the time
    • No cellular substrate or physiological role identified
    • Initially reported no RNA activity, later revised
  2. 2001 High

    Showed TREX1 acts as a homodimer with rigorous kinetics and defined substrate-structure preferences, framing its enzymatic mechanism.

    Evidence Purified mouse Trex1, steady-state kinetics, heparin competition assays

    PMID:11279105

    Open questions at the time
    • Dimer interface contributions to catalysis not yet resolved
    • No in vivo function
  3. 2004 High

    Demonstrated that TREX1 loss in vivo causes inflammatory disease rather than mutator/cancer phenotype, redirecting the field toward immune regulation.

    Evidence Trex1⁻/⁻ knockout mice, histopathology, survival analysis

    PMID:15254239

    Open questions at the time
    • Molecular trigger of inflammation unknown
    • Sensor pathway not yet identified
  4. 2006 High

    Linked TREX1 to human Aicardi-Goutieres syndrome and to a SET-complex apoptotic DNA-degradation role, connecting enzyme activity to disease and to genomic DNA clearance.

    Evidence AGS mutation activity assays, Trex1⁻/⁻ mice, reciprocal Co-IP with SET/NM23-H1, granzyme A cell death assays

    PMID:16818237 PMID:16845398

    Open questions at the time
    • Nature of the immune-triggering DNA species undefined
    • Relationship between apoptotic and homeostatic roles unclear
  5. 2007 High

    Provided crystal structures and the dominant-versus-recessive biochemical logic, explaining at atomic resolution why disease mutants lose activity and how dominant heterodimers poison wild-type enzyme.

    Evidence X-ray crystallography of substrate/product complexes, NMR, mutant homo/heterodimer activity assays, CA150 Co-IP

    PMID:17293595 PMID:17355961 PMID:17440703 PMID:18805785

    Open questions at the time
    • In vivo substrate still not pinned down
    • Functional relevance of CA150/proline-rich interactions untested
  6. 2007 High

    Defined TREX1 as an essential negative regulator of the ISD response that metabolizes reverse-transcribed and replication-derived ssDNA, establishing its homeostatic innate-immune function.

    Evidence Genetic ISD pathway analysis, Trex1⁻/⁻ cells, retroelement DNA accumulation, ER-to-nucleus relocalization after genotoxic stress

    PMID:18045533 PMID:18724932

    Open questions at the time
    • Downstream sensor not yet identified
    • Mechanism of stress-induced relocalization unresolved
  7. 2008 High

    Resolved the catalytic-impairment mechanism of disease mutants and identified catalytic metal/residue requirements, refining the structural enzymology.

    Evidence X-ray crystallography of apo and mutant TREX1, His124 mutagenesis, cation inhibition structures

    PMID:18780819 PMID:22071149

    Open questions at the time
    • Connection of structural defects to cellular phenotype indirect
  8. 2010 High

    Showed TREX1 degrades cytosolic HIV reverse-transcribed DNA to suppress type I IFN, extending its substrate range to retroviral DNA and revealing the TBK1/STING/IRF3 axis.

    Evidence Trex1⁻/⁻ cells, human T-cell/macrophage RNAi, Co-IP of TREX1 with HIV DNA, IFN induction assays

    PMID:20871604

    Open questions at the time
    • Whether degradation is direct on viral DNA or via accessory factors not fully resolved
  9. 2010 High

    Established stress-responsive and cytokine-responsive transcriptional control of TREX1 by AP-1, STAT1, and IFN-γ, embedding the enzyme in feedback regulation.

    Evidence Promoter reporters, EMSA, ChIP, c-fos/c-jun and STAT1 null cells

    PMID:20511593 PMID:21239708

    Open questions at the time
    • Quantitative contribution of induction to disease protection unmeasured
  10. 2011 High

    Dissected the dsDNA-degradation mechanism at endonuclease-generated 3' termini and the metal-dependent dominant-inhibition mechanism, explaining dominant autoimmune phenotypes.

    Evidence In vitro plasmid/chromatin dsDNA assays, mutant competition assays

    PMID:21808053 PMID:21937424

    Open questions at the time
    • In vivo source of endonuclease-generated termini unspecified
  11. 2012 High

    Revealed a type-I-IFN-independent arm of TREX1 deficiency involving lysosomal biogenesis, TFEB, and mTORC1, broadening its cellular impact beyond IFN.

    Evidence Trex1⁻/⁻ cells, STING/TBK1/IRF3/IRF7 epistasis, transcriptomics, lysosomal analysis

    PMID:23160154

    Open questions at the time
    • Mechanistic link between DNA clearance and lysosomal/mTORC1 changes unclear
  12. 2013 High

    Identified C-terminal ubiquitination and ubiquilin-1 binding as determinants of TREX1 subcellular localization, introducing post-translational control of trafficking.

    Evidence Deletion constructs, MS proteomics, Co-IP, co-localization

    PMID:23979357

    Open questions at the time
    • Physiological consequence of ubiquilin-1-directed punctae untested
    • Ubiquitin ligase unknown
  13. 2013 High

    Showed that oxidative DNA modification (8-OHG) renders DNA resistant to TREX1, providing a mechanism by which damaged self/pathogen DNA escapes clearance and activates cGAS-STING.

    Evidence In vitro degradation assays with oxidized DNA, cytosolic sensing assays

    PMID:23993650

    Open questions at the time
    • In vivo contribution of 8-OHG resistance to autoimmunity not quantified
  14. 2014 High

    Placed cGAS as the required sensor downstream of TREX1 loss both in cells and in vivo, defining the genetic backbone of TREX1-deficiency disease.

    Evidence TREX1/cGAS double-knockout cells and mice, ISG and autoantibody readouts

    PMID:24813208 PMID:26223655

    Open questions at the time
    • Tissue-specific drivers of inflammation not dissected
  15. 2014 Medium

    Defined a trans-acting dimer-interface residue (Arg62) and a PARP1 interaction, refining dimer catalysis and hinting at DNA-damage-response roles.

    Evidence Mutant heterodimer activity assays; MS, Co-IP, domain mapping for PARP1

    PMID:24616097 PMID:25278026

    Open questions at the time
    • PARP1 stabilization claim is partial/suggestive from a single lab
    • Functional role of nuclear TREX1-PARP1 interaction unresolved
  16. 2015 High

    Expanded TREX1 to an exoribonuclease, defined the dsDNA-unwinding mechanism in a knock-in lupus model, and uncovered the nuclease-independent OST-complex function, separating TREX1's two distinct activities.

    Evidence In vitro RNA assays, D18N-dsDNA crystal structure with D18N knock-in mice, OST Co-IP with frameshift mice/patient cells and aclacinomycin rescue

    PMID:25848017 PMID:25855793 PMID:26320659

    Open questions at the time
    • Physiological RNA substrates of TREX1 in vivo unidentified
    • How OST interaction integrates with nuclease function unclear
  17. 2017 Medium

    Established TREX1 as a cell-cycle-regulated (CDK1/Ser261) and radiation-induced enzyme that restrains tumor immunogenicity and suppresses L1 retrotransposons by a nuclease-independent ORF1p mechanism.

    Evidence MS phosphosite mapping with phosphomimetics, radiation dose-response with cGAS/STING readouts and tumor models, ORF1p Co-IP and L1 retrotransposition assays

    PMID:28297665 PMID:28334850 PMID:28598415

    Open questions at the time
    • L1 ORF1p depletion mechanism (single lab, two methods) needs reciprocal validation
    • Phosphatase identity at mitotic exit inferred not proven
  18. 2018 High

    Provided comprehensive structural views of TREX1 on branched DNA substrates showing capping and processive duplex digestion, and linked TREX1 to immunostimulatory exosome DNA cargo.

    Evidence Crystal structures of multiple TREX1-DNA complexes; exosome transfer and DC stimulation assays with TREX1 KD

    PMID:29734329 PMID:29907693

    Open questions at the time
    • Exosome study is medium-confidence single-lab; in vivo generality untested
  19. 2021 High

    Showed that ER tethering directs TREX1 to ruptured micronuclei and NE-rupture sites for DNA resection, that cGAS phase separation locally shields DNA from TREX1, and that nuclear TREX1 can itself drive DNA damage and cancer progression.

    Evidence Micronuclei purification with ER tracking and disease-mutant analysis, in vitro cGAS-DNA phase-separation reconstitution, live imaging of NE rupture in mammary duct models

    PMID:33476576 PMID:33606975 PMID:34551315

    Open questions at the time
    • How ER membrane gains access to ruptured envelopes mechanistically unresolved
    • Balance between protective resection and damaging nuclear activity context-dependent
  20. 2022 High

    Delivered the high-resolution human TREX1 structure with full disease-mutation mapping and assigned new substrate activities (NER sedDNA, 3'-PUA DNA-protein cross-links), broadening TREX1's role in DNA repair processing.

    Evidence Human apo (1.25 Å) and DNA-bound crystal structures; in vitro and cell assays with sedDNA; purified-enzyme DPC repair kinetics

    PMID:35349719 PMID:35357486 PMID:35879334

    Open questions at the time
    • In vivo importance of sedDNA/DPC processing for genome stability unquantified
    • Whether these activities feed cGAS signaling untested
  21. 2023 High

    Defined the structural/dynamic basis for TREX1 RNA discrimination and showed TREX1 clears virus-released cytoplasmic mitochondrial DNA to dampen antiviral sensing.

    Evidence Crystal structures, MD simulations and binding affinities on DNA/RNA junctions; CRISPR fitness screen and KO with mtDNA/cGAS-STING readouts during influenza infection

    PMID:37652009 PMID:37870446

    Open questions at the time
    • Generality of mtDNA clearance across other infections untested
  22. 2024 High

    Established TREX1 as a STAT1-driven negative-feedback brake on STING signaling exploitable for immunotherapy, and showed RVCL frameshift mutants mislocalize to the nucleus to impair HDR while TREX1 broadly limits CRISPR HDR template availability.

    Evidence Cancer-cell KO with in vivo tumor/PD-1 models; multi-organism (human/mouse/Drosophila) RVCL mutant HDR assays; genome-wide CRISPR HDR screen with protected-template rescue

    PMID:38227896 PMID:38824133 PMID:39134754

    Open questions at the time
    • Therapeutic window of TREX1 inhibition versus autoimmunity risk undefined
    • How nuclear mislocalization mechanistically blocks HDR not fully resolved

Open questions

Synthesis pass · forward-looking unresolved questions
  • How TREX1's distinct activities — cytosolic DNA/RNA degradation, ER-tethered micronuclear resection, nuclease-independent OST stabilization, and DNA-repair processing — are coordinated and prioritized in a single cell remains unresolved.
  • No unified model of how localization, PTMs, and partner interactions partition TREX1 between protective and pathogenic functions
  • Physiological RNA and DPC substrates in vivo unidentified
  • Mechanism coupling DNA clearance to lysosomal/mTORC1 program unclear

Mechanism profile

Synthesis pass · controlled-vocabulary classification · explore literature graph →
Molecular activity
GO:0140097 catalytic activity, acting on DNA 9 GO:0003677 DNA binding 5 GO:0016787 hydrolase activity 5 GO:0003723 RNA binding 2 GO:0140098 catalytic activity, acting on RNA 2
Localization
GO:0005634 nucleus 6 GO:0005783 endoplasmic reticulum 4 GO:0005829 cytosol 4
Pathway
R-HSA-168256 Immune System 8 R-HSA-1643685 Disease 4 R-HSA-73894 DNA Repair 4 R-HSA-8953897 Cellular responses to stimuli 4 R-HSA-5357801 Programmed Cell Death 2
Complex memberships
SET complexoligosaccharyltransferase (OST) complex

Evidence

Reading pass · 44 per-paper findings extracted from the source corpus
Year Finding Method Journal Conf PMIDs
1999 TREX1 encodes a 304-amino acid protein with three conserved motifs generating the exonuclease active site, most closely related to proofreading exonucleases of bacterial replicative DNA polymerases. Recombinant TREX1 expressed in E. coli is an active 3'→5' DNA exonuclease with greatest activity on partial duplex DNA containing 3' mismatched nucleotides; no activity was detected on single-stranded RNA or RNA-DNA partial duplexes. Recombinant protein expression in E. coli, in vitro exonuclease activity assays with ssDNA, partial duplex, and mispaired oligonucleotide substrates The Journal of biological chemistry High 10391904
2001 TREX1 (mouse) exists as a homodimer with robust 3'→5' exonuclease activity. Steady-state kinetics on ssDNA oligonucleotides yielded kcat ~22 s⁻¹ and KM ~19 nM for mouse Trex1. Competition assays with heparin and partial duplex DNA substrates established substrate structure preferences for 3' nucleotide excision. Recombinant protein expression and purification from E. coli, steady-state kinetic analysis, heparin competition assays The Journal of biological chemistry High 11279105
2004 Gene-targeted Trex1⁻/⁻ mice are viable but develop inflammatory myocarditis leading to cardiomyopathy and circulatory failure, establishing that TREX1 loss-of-function in vivo causes inflammatory disease rather than increased mutation frequency or cancer. Gene targeting (knockout mice), histopathology, survival analysis Molecular and cellular biology High 15254239
2006 TREX1 is the major mammalian 3'→5' DNA exonuclease encoded by AGS1; AGS-causing mutations abrogate TREX1 enzyme activity, and similar loss-of-function in Trex1⁻/⁻ mice leads to an inflammatory phenotype, implicating TREX1 in processing or clearing anomalous DNA structures to prevent innate immune triggering. Mutational analysis, exonuclease activity assays of patient-derived mutations, Trex1⁻/⁻ mouse model Nature genetics High 16845398
2006 TREX1 (but not TREX2) is a component of the endoplasmic reticulum-associated SET complex. TREX1 binds SET, colocalizes and translocates with the SET complex to the nucleus during granzyme A-mediated apoptosis, where it works in concert with NM23-H1 to degrade genomic DNA: after NM23-H1 nicks DNA, TREX1 removes nucleotides from the nicked 3' end. Silencing TREX1 inhibits DNA damage and cell death in response to perforin/granzyme A but not perforin/granzyme B. Co-immunoprecipitation, colocalization by immunofluorescence, siRNA knockdown, cell death assays with perforin/granzyme A treatment Molecular cell High 16818237
2007 Crystal structure of dimeric mouse TREX1 in substrate and product complexes with ssDNA and deoxyadenosine monophosphate reveals specific active-site interactions with the 3'-terminal nucleotide, providing molecular basis for specificity. Three AGS-associated TREX1 mutants show 4–35,000-fold reduced enzyme activity. The structure reveals an 8-amino acid polyproline II helix on the surface suggesting a mechanism for interactions with other protein complexes. X-ray crystallography, site-directed mutagenesis, in vitro exonuclease activity assays The Journal of biological chemistry High 17293595
2007 Crystal structures of dimeric murine TREX1 complexed with a nucleotide product and ssDNA substrate reveal sequence preferences for certain DNA motifs (GAG, ACA, CTGC). A conserved proline-rich region (PPPVPRPP) on the TREX1 surface interacts with WW2 domain of transcription elongation factor CA150, confirmed by NMR titration and co-immunoprecipitation with full-length TREX1. Nuclear localization of TREX1 requires loss of a C-terminal segment (leucine-rich repeat 3). X-ray crystallography, NMR titration, co-immunoprecipitation, confocal microscopy The Journal of biological chemistry High 17355961
2007 TREX1 acts in concert with the SET complex in granzyme A-mediated apoptosis. A heterozygous D18N mutation in TREX1 renders recombinant mutant TREX1 homodimers enzymatically inactive; wild-type/D18N heterodimers retain residual activity. Lymphoblastoid cells with D18N are significantly less sensitive to granzyme A-mediated cell death. Recombinant protein preparation, in vitro exonuclease activity assays, granzyme A cell death assays in patient-derived lymphoblastoid cells Journal of molecular medicine High 17440703
2007 TREX1 is an essential negative regulator of the IFN-stimulatory DNA (ISD) response. In Trex1-deficient cells, single-stranded DNA derived from endogenous retroelements accumulates. TREX1 can metabolize reverse-transcribed DNA, preventing chronic activation of innate immune signaling. Genetic pathway analysis links Trex1 deficiency to lethal autoimmunity through the ISD pathway. Genetic screen, Trex1⁻/⁻ mouse cells, ISD pathway reporter assays, DNA accumulation analysis, epistasis via Trex1 KO rescue experiments Cell High 18724932
2007 Dominant mutations in TREX1 (D200N and D18N) associated with autoimmune disease exhibit completely deficient dsDNA degradation activity while retaining partial ssDNA degradation activity. The dominant mutant heterodimers (WT/D200N and WT/D18N) inhibit the dsDNA degradation activity of WT TREX1 enzyme, explaining the dominant phenotype. The recessive R114H homodimer has dysfunctional dsDNA and ssDNA activities but does not inhibit WT enzyme. In vitro exonuclease activity assays with nicked dsDNA and ssDNA substrates using recombinant homo- and heterodimers The Journal of biological chemistry High 18805785
2007 Trex1, ordinarily associated with the endoplasmic reticulum, relocalizes to the S-phase nucleus after gamma irradiation or hydroxyurea treatment. Trex1-deficient cells show chronic ATM-dependent checkpoint activation even without exogenous stress, correlated with persistent ssDNA produced in S phase accumulating in the ER. Trex1 degrades ssDNA arising from aberrant replication intermediates to prevent chronic checkpoint signaling. Immunofluorescence/subcellular fractionation, gamma irradiation, hydroxyurea treatment, ATM inhibition, Trex1⁻/⁻ cells Cell High 18045533
2008 X-ray crystal structures of TREX1 apoprotein, dominant AGS/FCL mutants (D200H, D200N, D18N) and recessive mutant (V201D) reveal mechanism of catalytic impairment: D200H and D200N lose coordination of one active-site metal and the catalytic histidine (H195) is trapped in a non-catalytic conformation; D18N and V201D bind both metals but with inter-metal distances non-optimal for catalysis. All mutants show reduced mobility of catalytic histidine. X-ray crystallography of multiple mutant TREX1 proteins DNA repair High 22071149
2008 Lithium and sodium cations inhibit TREX1 exonuclease activity by competing with catalytic magnesium/manganese for the two metal-binding sites, inducing subtle active-site rearrangements. X-ray structures with Li⁺ and Na⁺ in complex with a nucleotide product explain this inhibition mechanism. Mutagenesis of His124 (highly conserved in DEDDh family) confirmed its requirement for TREX1 activity, identifying it as a catalytic residue. X-ray crystallography at 2.1 Å and 2.3 Å, in vitro exonuclease activity assays, site-directed mutagenesis of His124 Protein science High 18780819
2010 The cytosolic exonuclease TREX1 suppresses interferon triggered by HIV-1 infection. TREX1 binds cytosolic HIV DNA and degrades excess HIV DNA that would otherwise activate type I interferon via a TBK1/STING/IRF3-dependent pathway. In Trex1⁻/⁻ mouse cells and TREX1-knockdown human CD4⁺ T cells and macrophages, cytosolic HIV DNA accumulates and HIV infection induces type I interferon that inhibits HIV replication. Trex1⁻/⁻ mouse cells, siRNA knockdown in human T cells/macrophages, co-immunoprecipitation of TREX1 with HIV DNA, interferon induction assays Nature immunology High 20871604
2010 TREX1 expression is induced by genotoxic stress (UV light, benzo(a)pyrene, hydrogen peroxide) through an AP-1-dependent transcriptional mechanism. The mouse and human trex1 promoters contain AP-1 binding sites recognized by c-Fos and c-Jun; mutational inactivation of the AP-1 site abolishes induction. Upon genotoxic stress, TREX1 also translocates to the nucleus. TREX1-deficient cells show reduced recovery from UV/benzo(a)pyrene-induced replication inhibition. RT-PCR, promoter reporter assays, EMSA, c-fos/c-jun null cells, siRNA knockdown, immunofluorescence localization Nucleic acids research High 20511593
2011 TREX1 degrades plasmid and chromatin dsDNA by locating endonuclease-generated 3' termini. Dominant TREX1 mutants (D18N, D200N, D200H) retain intact DNA binding but have dysfunctional active-site chemistry; competition assays demonstrate that the dominant mutants block access of WT TREX1 to DNA 3' termini through metal-dependent ssDNA binding in catalytically inactive active sites. Residues Arg-174 and Lys-175 act with Arg-128 to facilitate dsDNA melting for ssDNA loading. In vitro exonuclease assays with plasmid and chromatin dsDNA, competition assays with dominant mutant and WT TREX1 proteins The Journal of biological chemistry High 21808053
2011 TREX1 R114H is a common AGS mutation; the R114H/R114H homodimer has 23-fold reduced ssDNA exonuclease activity. Compound heterodimers R114H/D201ins and R114H/A124ins exhibit 10-fold higher activity than the R114H homodimer, because Arg-114 residues from D201ins and A124ins protomers contribute to catalysis in the opposing R114H protomer across the dimer interface. Recombinant preparation of homo- and heterodimers, ssDNA and dsDNA exonuclease activity assays The Journal of biological chemistry High 21937424
2012 Trex1 regulates lysosomal biogenesis and activates a subset of interferon-stimulated genes (ISGs/antiviral genes) independently of type I interferon. This interferon-independent activation in Trex1-deficient cells requires STING, TBK1, IRF3, and IRF7. Trex1-deficient cells also have an expanded lysosomal compartment, altered subcellular localization of TFEB, and diminished mTORC1 activity. Trex1⁻/⁻ mouse cells, STING/TBK1/IRF3/IRF7 genetic epistasis, transcriptomic profiling, lysosomal compartment analysis Nature immunology High 23160154
2013 TREX1 C-terminal region controls cellular localization through ubiquitination. A highly conserved sequence in the CTR is required for ubiquitination at multiple lysine residues through a non-canonical ubiquitin linkage. Ubiquilin 1 was identified by proteomics as a TREX1 CTR-interacting protein (confirmed in vitro and in vivo), and ubiquilin 1 localizes TREX1 to cytosolic punctate structures dependent on the TREX1 CTR and catalytic core lysines. Disease-causing catalytically competent TREX1 mutants show differential ubiquitination and altered ubiquilin 1 co-localization. Deletion construct transfection, mass spectrometry proteomics, co-immunoprecipitation, in vitro binding assay, cotransfection co-localization The Journal of biological chemistry High 23979357
2013 Oxidized DNA containing 8-hydroxyguanosine (8-OHG) is resistant to TREX1-mediated degradation. This resistance potentiates cGAS/STING-dependent cytosolic immune recognition, as oxidative modifications reduce TREX1's ability to degrade the DNA. 8-OHG arises in pathogen DNA during lysosomal ROS exposure and in NET DNA during oxidative burst. In vitro TREX1 degradation assays with oxidized vs. unmodified DNA, cytosolic DNA sensing assays, cell-based immune activation experiments Immunity High 23993650
2014 TREX1 deficiency triggers cell-autonomous innate immune activation that is completely dependent on cGAS. Knockout of cGAS in TREX1-deficient cells abrogates spontaneous induction of interferon-stimulated genes, placing cGAS upstream of the type I IFN response in TREX1-deficient settings. TREX1/cGAS double-knockout cells, ISG expression assays Journal of immunology High 24813208
2014 TREX1 directly interacts with PARP1 via PARP1's N-terminal zinc finger domains. This interaction occurs after nuclear translocation of TREX1 in response to DNA damage. TREX1 may contribute to stabilization of PARP1 levels and its activity during the DNA damage response. Mass spectrometry, co-immunoprecipitation assays, in vivo overexpression, domain mapping The Journal of biological chemistry Medium 25278026
2015 TREX1 is also an exoribonuclease that degrades single-stranded RNA but not double-stranded RNA. The AGS disease mutant TREX1-D200N is defective in RNA degradation. TREX1 activity is inhibited by stretches of pyrimidine residues; TREX1 is active on native tRNA substrates. The substrate-binding site is open enough to accommodate the 2'-OH of RNA. In vitro exoribonuclease assays with purified TREX1, kinetic measurements, mutant protein analysis, structural analysis of published crystal structures The Journal of biological chemistry High 25855793
2015 Crystal structure of TREX1 D18N in complex with dsDNA reveals a novel DNA-unwinding mechanism where TREX1 uses specific residues to separate polynucleotide strands for ssDNA loading into the active site. TREX1 D18N has dysfunctional dsDNA-degrading activity. TREX1 D18N knock-in mice develop systemic lupus-like inflammation with autoantibodies to dsDNA and immune complex deposition, demonstrating that dysfunctional dsDNA degradation alone is sufficient to cause lupus-like disease. X-ray crystallography of TREX1 D18N-dsDNA complex, knock-in mouse model, immunohistochemistry, autoantibody assays Proceedings of the National Academy of Sciences High 25848017
2015 TREX1 C terminus suppresses immune activation independently of nuclease activity by interacting with the ER oligosaccharyltransferase (OST) complex and stabilizing its catalytic integrity. C-terminal frameshift mutations (as in RVCL) dysregulate the OST complex leading to free glycan release from dolichol carriers and immune activation. OST inhibition with aclacinomycin corrects glycan and immune defects in TREX1 frameshift mutant cells and mice. Co-immunoprecipitation of TREX1 with OST complex, glycan analysis, TREX1 KO and frameshift knock-in mice, patient lymphoblasts, aclacinomycin pharmacological rescue Immunity High 26320659
2015 cGAS is required for lethal autoimmune disease in Trex1⁻/⁻ mice. Trex1⁻/⁻ cGAS⁻/⁻ double knockout mice are completely protected from lethality, show dramatically reduced tissue inflammation, and fail to develop autoantibodies, placing cGAS as a key driver of Trex1-deficiency-associated autoimmune disease. Double-knockout mouse genetics, histopathology, survival analysis, autoantibody measurement Journal of immunology High 26223655
2017 DNA exonuclease TREX1 is induced by radiation doses above 12–18 Gy in cancer cells and attenuates immunogenicity by degrading cytosolic double-stranded DNA that accumulates upon radiation. Cytosolic DNA stimulates IFN-β secretion via cGAS and STING activation. At doses that do not induce TREX1, repeated irradiation amplifies IFN-β production, drives BATF3-dependent DC recruitment and CD8⁺ T cell priming for abscopal tumor rejection. Radiation dose–response experiments in cancer cells, siRNA knockdown, cGAS/STING pathway reporter assays, mouse tumor models, T cell depletion experiments Nature communications High 28598415
2017 TREX1 suppresses L1 retrotransposon activity through a nuclease-independent mechanism. TREX1 interacts with L1 ORF1p protein and alters its intracellular localization, triggering ORF1p depletion and reducing L1-mediated nicking of genomic DNA. AGS-associated TREX1 mutants competent for DNA exonuclease activity are deficient in inducing ORF1p depletion. Co-immunoprecipitation, immunofluorescence co-localization, L1 retrotransposition and genomic nicking assays, AGS mutant analysis Nucleic acids research Medium 28334850
2017 During mitosis, TREX1 is predominantly phosphorylated at C-terminal Serine-261 by Cyclin B/CDK1, and dephosphorylated at mitotic exit likely by PP1/PP2-type phosphatases. Mitotic phosphorylation does not affect DNase activity but phosphomimetic mutations disrupt TREX1 interaction with OST subunit RPN1 and elevate a glyco-gene expression signature. Thus cell-cycle-dependent phosphorylation regulates TREX1's OST-interacting function. Mass spectrometry identification of phosphorylation site, CDK1 inhibition, phosphomimetic mutant co-immunoprecipitation with RPN1, RNA-seq of Trex1⁻/⁻ MEFs with WT or phospho-mutants Cell reports High 28297665
2017 TREX1 transcription is induced by IFN-γ in primary macrophages via STAT1 binding to IFN-γ activation site (GAS) boxes in the Trex1 promoter, and c-Jun (but not c-Fos, JunB, or CREB) binding to an AP-1 box. This was confirmed using STAT1 KO macrophages and EMSA/ChIP assays. EMSA, ChIP, transient transfection reporter assays, STAT1 KO macrophages, promoter mutational analysis Journal of immunology High 21239708
2018 Crystal structures of TREX1 complexed with various DNA substrates (L-shaped, Y-shaped DNA) reveal that the Leu24-Pro25-Ser26 cluster serves to cap the non-scissile 5'-end for precise removal of short 3'-overhangs or to wedge into dsDNA for further digestion along the duplex. Arg128 shows consistent interaction patterns with the non-scissile strand across all structures. TREX1 can degrade dsDNA to completion. X-ray crystallography of multiple TREX1-DNA complexes, in vitro biochemical dsDNA degradation assays PLoS biology High 29734329
2018 Tumor-derived exosomes from irradiated cancer cells (RT-TEX) transfer dsDNA to dendritic cells and stimulate STING-dependent IFN-β production. The IFN-stimulatory dsDNA cargo of RT-TEX is regulated by TREX1 expression in the parent cancer cells, demonstrating that TREX1 controls the immunostimulatory DNA content packaged into exosomes. Exosome purification and characterization, TREX1 knockdown in cancer cells, DC stimulation assays, in vivo tumor vaccination experiments Cancer immunology research Medium 29907693
2021 cGAS-DNA phase separation inhibits TREX1-mediated DNA degradation. cGAS phase separation forms a selective environment that suppresses TREX1 catalytic function and restricts DNA degradation to the droplet periphery. An AGS-associated TREX1 mutation increases TREX1 penetration into the repressive droplet interior, specifically impairing degradation of phase-separated DNA. In vitro phase separation reconstitution, TREX1 activity assays on phase-separated DNA, confocal imaging of droplets, disease mutant analysis Molecular cell High 33606975
2021 The ER-associated nuclease TREX1 inhibits cGAS activation at micronuclei by degrading micronuclear DNA upon micronuclear envelope rupture. The ER accesses ruptured micronuclei enabling TREX1 nucleolytic attack. TREX1 mutations that cause immune disease untether TREX1 from the ER, disrupt localization to micronuclei, diminish micronuclear DNA damage, and enhance cGAS activation. ER tethering is thus mechanistically required for TREX1's role in preventing innate immune activation at micronuclei. Micronuclei purification, TREX1 localization by immunofluorescence, ER membrane tracking, disease mutant analysis, cGAS activation assays, functional nuclease activity at micronuclei Molecular cell High 33476576
2021 TREX1 translocates into the nucleus after nuclear envelope rupture and is required to induce DNA damage in this context. Inside the mammary duct, cellular crowding leads to nuclear envelope ruptures that generate TREX1-dependent DNA damage, driving progression of in situ carcinoma to the invasive stage. Live-cell imaging, TREX1 nuclear translocation assays after NE rupture, TREX1 KO/knockdown, in vivo mammary duct compression models, DNA damage markers Cell High 34551315
2022 Crystal structures of human TREX1 in apo (1.25 Å) and DNA-bound (2.2 Å) conformations establish complete solvation of the exonuclease active site and identify specific substitutions involved in DNA recognition. Mapping of all human disease-associated mutations identifies distinct categories affecting enzymatic function, protein stability, and interaction with cGAS-DNA liquid droplets. X-ray crystallography at 1.25 Å (apo) and 2.2 Å (DNA complex), comprehensive mutation mapping Nature communications High 35879334
2022 TREX1 degrades the 3' end of the small excised damage-containing DNA oligonucleotides (~30 nt, sedDNA) produced by nucleotide excision repair. TREX1 knockdown increases sedDNA levels and their association with NER proteins TFIIH and RPA; overexpression of WT but not nuclease-inactive TREX1 diminishes sedDNA levels. Purified TREX1 efficiently degrades DNA 3' of UV photoproducts in sedDNA. TREX1 modulation does not affect NER efficiency or cell survival. RNAi screen, TREX1 knockdown/overexpression in UV-irradiated cells, in vitro assays with purified TREX1 and synthetic sedDNA substrates Nucleic acids research High 35357486
2022 Purified human TREX1 repairs 3'-PUA-peptide/protein DNA-protein cross-links (DPCs) arising from abasic sites, acting as an exonuclease on the 3'-PUA-modified terminus. TREX1 can directly repair 3'-PUA-histone DPCs but not 3'-PUA-PARP1 DPCs unless proteolysis occurs first. Kinetic constants were determined for DPC repair by TREX1. In vitro enzymatic assays with purified recombinant TREX1 and synthetic 3'-PUA-peptide adducts, kinetic analysis, histone and PARP1 DPC substrates Nucleic acids research High 35349719
2023 TREX1 can digest ssRNA and DNA/RNA hybrids but not dsRNA. Crystal structures of TREX1-RNA product and TREX1-nucleotide complexes show 2'-OH does not impose steric hindrance for RNA recognition. MD simulations and binding affinity measurements with synthetic DNA-RNA junctions demonstrate that 2'-OH-mediated intra-chain hydrogen bonding in RNA increases conformational rigidity, reducing TREX1 binding affinity and catalytic cleavage efficiency. In vitro exonuclease activity assays, X-ray crystallography, all-atom molecular dynamics simulations, binding affinity measurements Nucleic acids research High 37870446
2023 TREX1 degrades cytoplasmic mitochondrial DNA (mtDNA) released during influenza virus infection, preventing cGAS/STING-mediated antiviral sensing. Infection triggers release of mtDNA into the cytoplasm; TREX1 metabolizes this self-DNA, thereby preventing amplification of antiviral signaling and thus aiding viral replication. CRISPR-based genome-wide fitness screen during influenza infection, TREX1 KO cells, mtDNA accumulation assays, cGAS/STING pathway reporters Cell host & microbe High 37652009
2024 TREX1 expression is coordinately induced with STING by autocrine IFN and downstream STAT1 in cancer cells, acting as a negative feedback mechanism. TREX1 restrains STING-dependent nucleic acid sensing via its catalytic DNA-degrading function. TREX1 inactivation in cancer cells unleashes STING-IFN signaling, recruiting T cells and NK cells and cooperating with PD-1 blockade. Cancer cell TREX1 KO/knockdown, STING pathway reporter assays, in vivo mouse tumor models with immune cell depletion, RNA-seq, combination immunotherapy experiments Cancer discovery High 38227896
2024 TREX1 C-terminal frameshift variants associated with RVCL mislocalize to the nucleus (due to deletion of the ER-anchoring domain) and inhibit homology-directed repair (HDR), causing DNA deletions. RVCL TREX1 mutants increase cellular vulnerability to DNA damage induced by chemotherapy and cytokines, and cells expressing these mutants are depleted in vivo. Mechanistic studies in Drosophila, mice, and human cells confirm nuclear TREX1 causes DNA damage and disrupts HDR. RVCL patient cell analysis, mouse models, Drosophila genetics, CRISPR HDR assays, DNA damage marker analysis, PARP inhibitor sensitivity assays Nature communications High 38824133
2024 TREX1 degrades single-stranded and linearized double-stranded DNA repair templates used for CRISPR-Cas9-mediated HDR, reducing HDR efficiency. TREX1 expression level serves as a biomarker for HDR efficiency across cell types. TREX1 knockout or use of chemically protected ssDNA templates rescues HDR efficiency by 2- to 8-fold in TREX1-expressing cells including primary T cells and hematopoietic stem/progenitor cells. Genome-wide CRISPR screen for HDR suppressors in Fanconi anemia lymphoblasts, TREX1 KO in multiple cell types, HDR efficiency assays with protected vs. unprotected templates Nature biotechnology High 39134754
2014 Arg-62 residues of TREX1 extend across the dimer interface into the active site of the opposing protomer to coordinate substrate DNA and affect catalysis in trans. The TREX1(R62A/R62A) homodimer exhibits ~50-fold reduced ssDNA and dsDNA degradation. Dominant mutants (D18H, D18N, D200H, D200N) compound heterodimers with R62A in the opposing protomer show higher activity than the corresponding homodimers, demonstrating that Arg-62 provides structural elements required for full catalytic activity in the opposing protomer. Site-directed mutagenesis, recombinant homo- and heterodimer preparation, in vitro ssDNA and dsDNA exonuclease activity assays The Journal of biological chemistry High 24616097

Source papers

Stage 0 corpus · 100 papers · ranked by NIH iCite citations
Year Title Journal Citations PMID
2017 DNA exonuclease Trex1 regulates radiotherapy-induced tumour immunogenicity. Nature communications 1467 28598415
2008 Trex1 prevents cell-intrinsic initiation of autoimmunity. Cell 1061 18724932
2006 Mutations in the gene encoding the 3'-5' DNA exonuclease TREX1 cause Aicardi-Goutières syndrome at the AGS1 locus. Nature genetics 749 16845398
2007 Mutations in the gene encoding the 3'-5' DNA exonuclease TREX1 are associated with systemic lupus erythematosus. Nature genetics 565 17660818
2007 Trex1 exonuclease degrades ssDNA to prevent chronic checkpoint activation and autoimmune disease. Cell 481 18045533
2010 The cytosolic exonuclease TREX1 inhibits the innate immune response to human immunodeficiency virus type 1. Nature immunology 436 20871604
2013 Oxidative damage of DNA confers resistance to cytosolic nuclease TREX1 degradation and potentiates STING-dependent immune sensing. Immunity 394 23993650
2013 Assessment of interferon-related biomarkers in Aicardi-Goutières syndrome associated with mutations in TREX1, RNASEH2A, RNASEH2B, RNASEH2C, SAMHD1, and ADAR: a case-control study. The Lancet. Neurology 371 24183309
2004 Gene-targeted mice lacking the Trex1 (DNase III) 3'-->5' DNA exonuclease develop inflammatory myocarditis. Molecular and cellular biology 328 15254239
2015 Cutting Edge: cGAS Is Required for Lethal Autoimmune Disease in the Trex1-Deficient Mouse Model of Aicardi-Goutières Syndrome. Journal of immunology (Baltimore, Md. : 1950) 324 26223655
2007 Heterozygous mutations in TREX1 cause familial chilblain lupus and dominant Aicardi-Goutieres syndrome. American journal of human genetics 308 17357087
2018 Exosomes Shuttle TREX1-Sensitive IFN-Stimulatory dsDNA from Irradiated Cancer Cells to DCs. Cancer immunology research 307 29907693
2017 Modeling of TREX1-Dependent Autoimmune Disease using Human Stem Cells Highlights L1 Accumulation as a Source of Neuroinflammation. Cell stem cell 262 28803918
1999 Identification and expression of the TREX1 and TREX2 cDNA sequences encoding mammalian 3'-->5' exonucleases. The Journal of biological chemistry 242 10391904
2011 Evaluation of the TREX1 gene in a large multi-ancestral lupus cohort. Genes and immunity 220 21270825
2006 The exonuclease TREX1 is in the SET complex and acts in concert with NM23-H1 to degrade DNA during granzyme A-mediated cell death. Molecular cell 219 16818237
2014 TREX1 deficiency triggers cell-autonomous immunity in a cGAS-dependent manner. Journal of immunology (Baltimore, Md. : 1950) 216 24813208
2021 Compromised nuclear envelope integrity drives TREX1-dependent DNA damage and tumor cell invasion. Cell 209 34551315
2017 A prosurvival DNA damage-induced cytoplasmic interferon response is mediated by end resection factors and is limited by Trex1. Genes & development 191 28279982
2007 A mutation in TREX1 that impairs susceptibility to granzyme A-mediated cell death underlies familial chilblain lupus. Journal of molecular medicine (Berlin, Germany) 180 17440703
2021 cGAS phase separation inhibits TREX1-mediated DNA degradation and enhances cytosolic DNA sensing. Molecular cell 172 33606975
2015 Human disease phenotypes associated with mutations in TREX1. Journal of clinical immunology 171 25731743
2021 ER-directed TREX1 limits cGAS activation at micronuclei. Molecular cell 166 33476576
2015 Exonuclease TREX1 degrades double-stranded DNA to prevent spontaneous lupus-like inflammatory disease. Proceedings of the National Academy of Sciences of the United States of America 151 25848017
2001 Excision of 3' termini by the Trex1 and TREX2 3'-->5' exonucleases. Characterization of the recombinant proteins. The Journal of biological chemistry 139 11279105
2012 Trex1 regulates lysosomal biogenesis and interferon-independent activation of antiviral genes. Nature immunology 127 23160154
2018 Inhibition of Cyclic GMP-AMP Synthase Using a Novel Antimalarial Drug Derivative in Trex1-Deficient Mice. Arthritis & rheumatology (Hoboken, N.J.) 115 29781188
2008 The TREX1 double-stranded DNA degradation activity is defective in dominant mutations associated with autoimmune disease. The Journal of biological chemistry 115 18805785
2007 The crystal structure of TREX1 explains the 3' nucleotide specificity and reveals a polyproline II helix for protein partnering. The Journal of biological chemistry 103 17293595
2008 New roles for the major human 3'-5' exonuclease TREX1 in human disease. Cell cycle (Georgetown, Tex.) 100 18583934
2017 Immune Diseases Associated with TREX1 and STING Dysfunction. Journal of interferon & cytokine research : the official journal of the International Society for Interferon and Cytokine Research 92 28475463
2015 Cytosolic Nuclease TREX1 Regulates Oligosaccharyltransferase Activity Independent of Nuclease Activity to Suppress Immune Activation. Immunity 90 26320659
2024 TREX1 Inactivation Unleashes Cancer Cell STING-Interferon Signaling and Promotes Antitumor Immunity. Cancer discovery 86 38227896
2019 cGAS activation causes lupus-like autoimmune disorders in a TREX1 mutant mouse model. Journal of autoimmunity 78 30872080
2016 Loss of Trex1 in Dendritic Cells Is Sufficient To Trigger Systemic Autoimmunity. Journal of immunology (Baltimore, Md. : 1950) 73 27511730
2017 Aicardi-Goutières syndrome protein TREX1 suppresses L1 and maintains genome integrity through exonuclease-independent ORF1p depletion. Nucleic acids research 72 28334850
2019 Assessment of Clinical Response to Janus Kinase Inhibition in Patients With Familial Chilblain Lupus and TREX1 Mutation. JAMA dermatology 68 30673078
2011 Dominant mutation of the TREX1 exonuclease gene in lupus and Aicardi-Goutieres syndrome. The Journal of biological chemistry 65 21808053
2001 Structure and expression of the TREX1 and TREX2 3' --> 5' exonuclease genes. The Journal of biological chemistry 64 11278605
2010 Three prime exonuclease I (TREX1) is Fos/AP-1 regulated by genotoxic stress and protects against ultraviolet light and benzo(a)pyrene-induced DNA damage. Nucleic acids research 60 20511593
2014 Identification of a pathogenic variant in TREX1 in early-onset cerebral systemic lupus erythematosus by Whole-exome sequencing. Arthritis & rheumatology (Hoboken, N.J.) 59 25138095
2021 TREX1 as a Novel Immunotherapeutic Target. Frontiers in immunology 56 33868310
2009 Biochemical properties of mammalian TREX1 and its association with DNA replication and inherited inflammatory disease. Biochemical Society transactions 50 19442247
2022 Structural basis of human TREX1 DNA degradation and autoimmune disease. Nature communications 49 35879334
2016 MicroRNA regulation of endothelial TREX1 reprograms the tumour microenvironment. Nature communications 49 27886180
2014 The 3'-5' DNA exonuclease TREX1 directly interacts with poly(ADP-ribose) polymerase-1 (PARP1) during the DNA damage response. The Journal of biological chemistry 49 25278026
2020 TREX1 - Apex predator of cytosolic DNA metabolism. DNA repair 48 32615442
2007 Structure of the dimeric exonuclease TREX1 in complex with DNA displays a proline-rich binding site for WW Domains. The Journal of biological chemistry 48 17355961
2014 TREX1 C-terminal frameshift mutations in the systemic variant of retinal vasculopathy with cerebral leukodystrophy. Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology 47 25213617
2013 The exonuclease Trex1 restrains macrophage proinflammatory activation. Journal of immunology (Baltimore, Md. : 1950) 47 24218451
2023 Pathogen-driven CRISPR screens identify TREX1 as a regulator of DNA self-sensing during influenza virus infection. Cell host & microbe 42 37652009
2011 The TREX1 exonuclease R114H mutation in Aicardi-Goutières syndrome and lupus reveals dimeric structure requirements for DNA degradation activity. The Journal of biological chemistry 42 21937424
2024 The Exonuclease TREX1 Constitutes an Innate Immune Checkpoint Limiting cGAS/STING-Mediated Antitumor Immunity. Cancer immunology research 39 38489753
2015 Human DNA Exonuclease TREX1 Is Also an Exoribonuclease That Acts on Single-stranded RNA. The Journal of biological chemistry 39 25855793
2013 The TREX1 C-terminal region controls cellular localization through ubiquitination. The Journal of biological chemistry 39 23979357
2018 Structural basis for overhang excision and terminal unwinding of DNA duplexes by TREX1. PLoS biology 38 29734329
2010 A de novo p.Asp18Asn mutation in TREX1 in a patient with Aicardi-Goutières syndrome. American journal of medical genetics. Part A 38 20799324
2018 HIV-1 Activation of Innate Immunity Depends Strongly on the Intracellular Level of TREX1 and Sensing of Incomplete Reverse Transcription Products. Journal of virology 35 29769349
2017 Lack of Trex1 Causes Systemic Autoimmunity despite the Presence of Antiretroviral Drugs. Journal of immunology (Baltimore, Md. : 1950) 35 28835460
2009 TREX1 acts in degrading damaged DNA from drug-treated tumor cells. DNA repair 35 19617005
2024 Intratumoral TREX1 Induction Promotes Immune Evasion by Limiting Type I IFN. Cancer immunology research 33 38408184
2013 Rare variants in the TREX1 gene and susceptibility to autoimmune diseases. BioMed research international 31 24224166
2017 DNase-active TREX1 frame-shift mutants induce serologic autoimmunity in mice. Journal of autoimmunity 30 28325644
2013 Human three prime exonuclease TREX1 is induced by genotoxic stress and involved in protection of glioma and melanoma cells to anticancer drugs. Biochimica et biophysica acta 29 23578789
2013 A nationwide survey of Aicardi-Goutières syndrome patients identifies a strong association between dominant TREX1 mutations and chilblain lesions: Japanese cohort study. Rheumatology (Oxford, England) 29 24300241
2022 Human TDP1, APE1 and TREX1 repair 3'-DNA-peptide/protein cross-links arising from abasic sites in vitro. Nucleic acids research 28 35349719
2023 Suppression of TREX1 deficiency-induced cellular senescence and interferonopathies by inhibition of DNA damage response. iScience 27 37416470
2015 Typing TREX1 gene in patients with systemic lupus erythematosus. Reumatismo 27 26150267
2013 A homozygous NOTCH3 mutation p.R544C and a heterozygous TREX1 variant p.C99MfsX3 in a family with hereditary small vessel disease of the brain. Journal of the Chinese Medical Association : JCMA 27 23602593
2011 Defects in DNA degradation revealed in crystal structures of TREX1 exonuclease mutations linked to autoimmune disease. DNA repair 27 22071149
2019 TREX1 As a Potential Therapeutic Target for Autoimmune and Inflammatory Diseases. Current pharmaceutical design 26 31475890
2014 Safeguard against DNA sensing: the role of TREX1 in HIV-1 infection and autoimmune diseases. Frontiers in microbiology 26 24817865
2022 TREX1 degrades the 3' end of the small DNA oligonucleotide products of nucleotide excision repair in human cells. Nucleic acids research 24 35357486
2019 T Cells Produce IFN-α in the TREX1 D18N Model of Lupus-like Autoimmunity. Journal of immunology (Baltimore, Md. : 1950) 24 31826941
2022 Cytoplasmic DNA in cancer cells: Several pathways that potentially limit DNase2 and TREX1 activities. Biochimica et biophysica acta. Molecular cell research 23 35489653
2021 Photosensitivity and cGAS-Dependent IFN-1 Activation in Patients with Lupus and TREX1 Deficiency. The Journal of investigative dermatology 23 34400195
2024 Inherited C-terminal TREX1 variants disrupt homology-directed repair to cause senescence and DNA damage phenotypes in Drosophila, mice, and humans. Nature communications 22 38824133
2019 Three Prime Repair Exonuclease 1 (TREX1) expression correlates with cervical cancer cells growth in vitro and disease progression in vivo. Scientific reports 22 30674977
2017 Mitotic Phosphorylation of TREX1 C Terminus Disrupts TREX1 Regulation of the Oligosaccharyltransferase Complex. Cell reports 22 28297665
2011 Characterization of Trex1 induction by IFN-γ in murine macrophages. Journal of immunology (Baltimore, Md. : 1950) 22 21239708
2008 Structural and biochemical studies of TREX1 inhibition by metals. Identification of a new active histidine conserved in DEDDh exonucleases. Protein science : a publication of the Protein Society 22 18780819
2024 MRE11 and TREX1 control senescence by coordinating replication stress and interferon signaling. Nature communications 21 38926338
2020 Celastrol ameliorates autoimmune disorders in Trex1-deficient mice. Biochemical pharmacology 21 32565148
2017 TREX1 Cuts Down on Cancer Immunogenicity. Trends in cell biology 21 28625463
2014 TLR ligands up-regulate Trex1 expression in murine conventional dendritic cells through type I Interferon and NF-κB-dependent signaling pathways. Journal of leukocyte biology 21 24598055
2008 TREX1 DNA exonuclease deficiency, accumulation of single stranded DNA and complex human genetic disorders. DNA repair 21 18406216
2024 Targeting TREX1 Induces Innate Immune Response in Drug-Resistant Small-Cell Lung Cancer. Cancer research communications 20 39177280
2023 TREX1 cytosolic DNA degradation correlates with autoimmune disease and cancer immunity. Clinical and experimental immunology 20 36745566
2019 TREX1 variants in Sjogren's syndrome related lymphomagenesis. Cytokine 20 31326279
2024 Removal of TREX1 activity enhances CRISPR-Cas9-mediated homologous recombination. Nature biotechnology 19 39134754
2021 Type I interferonopathies with novel compound heterozygous TREX1 mutations in two siblings with different symptoms responded to tofacitinib. Pediatric rheumatology online journal 19 33407657
2022 TREX1 plays multiple roles in human diseases. Cellular immunology 18 35468328
2018 TREX1 Mutation Causing Autosomal Dominant Thrombotic Microangiopathy and CKD-A Novel Presentation. American journal of kidney diseases : the official journal of the National Kidney Foundation 18 29941221
2018 TREX1 D18N mice fail to process erythroblast DNA resulting in inflammation and dysfunctional erythropoiesis. Autoimmunity 18 30422000
2023 Molecular insight into the specific enzymatic properties of TREX1 revealing the diverse functions in processing RNA and DNA/RNA hybrids. Nucleic acids research 17 37870446
2023 TREX1 is required for microglial cholesterol homeostasis and oligodendrocyte terminal differentiation in human neural assembloids. Molecular psychiatry 16 38129659
2019 Measuring TREX1 and TREX2 exonuclease activities. Methods in enzymology 16 31455522
2018 TREX1 is expressed by microglia in normal human brain and increases in regions affected by ischemia. Brain pathology (Zurich, Switzerland) 16 30062819
2007 TREX1 polymorphisms associated with autoantibodies in patients with systemic lupus erythematosus. Rheumatology international 16 18092167
2014 The Arg-62 residues of the TREX1 exonuclease act across the dimer interface contributing to catalysis in the opposing protomers. The Journal of biological chemistry 15 24616097

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