Trex1 three prime repair exonuclease 1 [Mus musculus (house mouse)] - Gene

Summary

Official Symbol: Trex1provided by MGI
Official Full Name: three prime repair exonuclease 1provided by MGI
Primary source: MGI:MGI:1328317
See related: Ensembl:ENSMUSG00000049734 AllianceGenome:MGI:1328317
Gene type: protein coding
RefSeq status: VALIDATED
Organism: Mus musculus
Lineage: Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Glires; Rodentia; Myomorpha; Muroidea; Muridae; Murinae; Mus; Mus
Summary: Enables several functions, including DNA binding activity; WW domain binding activity; and magnesium ion binding activity. Involved in DNA metabolic process. Acts upstream of or within several processes, including DNA metabolic process; circulatory system development; and regulation of biosynthetic process. Located in cytosol; endoplasmic reticulum; and nuclear replication fork. Part of oligosaccharyltransferase complex and protein-DNA complex. Is expressed in lens. Used to study Aicardi-Goutieres syndrome and cutaneous lupus erythematosus. Human ortholog(s) of this gene implicated in Aicardi-Goutieres syndrome; Chilblain lupus; retinal vasculopathy with cerebral leukodystrophy; and systemic lupus erythematosus. Orthologous to human TREX1 (three prime repair exonuclease 1). [provided by Alliance of Genome Resources, Dec 2024]
Expression: Ubiquitous expression in spleen adult (RPKM 79.9), mammary gland adult (RPKM 65.5) and 27 other tissues See more
Orthologs: human all
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Genomic context

Location:: 9 F2; 9 59.63 cM

Exon count:: 3

Annotation release	Status	Assembly	Chr	Location
RS_2024_02	current	GRCm39 (GCF_000001635.27)	9	NC_000075.7 (108887000..108888791, complement)
108.20200622	previous assembly	GRCm38.p6 (GCF_000001635.26)	9	NC_000075.6 (109057932..109059723, complement)

Chromosome 9 - NC_000075.7 Genomic Context describing neighboring genes

Genomic regions, transcripts, and products

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Go to reference sequence details

Genomic Sequence:

Go to nucleotide: Graphics FASTA GenBank

Expression

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See details

Project title: Mouse ENCODE transcriptome data
Description: RNA profiling data sets generated by the Mouse ENCODE project.
BioProject: PRJNA66167
Publication: PMID 25409824
Analysis date: n/a

Bibliography

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GeneRIFs: Gene References Into Functions

What's a GeneRIF?

cGAS activation in classical dendritic cells causes autoimmunity in TREX1-deficient mice.
Title: cGAS activation in classical dendritic cells causes autoimmunity in TREX1-deficient mice.
Intratumoral TREX1 Induction Promotes Immune Evasion by Limiting Type I IFN.
Title: Intratumoral TREX1 Induction Promotes Immune Evasion by Limiting Type I IFN.
The Exonuclease TREX1 Constitutes an Innate Immune Checkpoint Limiting cGAS/STING-Mediated Antitumor Immunity.
Title: The Exonuclease TREX1 Constitutes an Innate Immune Checkpoint Limiting cGAS/STING-Mediated Antitumor Immunity.
Inherited C-terminal TREX1 variants disrupt homology-directed repair to cause senescence and DNA damage phenotypes in Drosophila, mice, and humans.
Title: Inherited C-terminal TREX1 variants disrupt homology-directed repair to cause senescence and DNA damage phenotypes in Drosophila, mice, and humans.
Molecular insight into the specific enzymatic properties of TREX1 revealing the diverse functions in processing RNA and DNA/RNA hybrids.
Title: Molecular insight into the specific enzymatic properties of TREX1 revealing the diverse functions in processing RNA and DNA/RNA hybrids.
Targeting Bcl6 in the TREX1 D18N murine model ameliorates autoimmunity by modulating T-follicular helper cells and germinal center B cells.
Title: Targeting Bcl6 in the TREX1 D18N murine model ameliorates autoimmunity by modulating T-follicular helper cells and germinal center B cells.
these findings demonstrate that TREX1 enzymatic activity is crucial to prevent inappropriate DNA sensing and IFN type I production in immune cells
Title: T Cells Produce IFN-α in the TREX1 D18N Model of Lupus-like Autoimmunity.
These results indicate that cGAS is a key mediator in the autoimmune disease associated with defective TREX1 function, providing additional insights into disease pathogenesis and guidance to the development of therapeutics for human systemic autoimmune disorders.
Title: cGAS activation causes lupus-like autoimmune disorders in a TREX1 mutant mouse model.
Leu24-Pro25-Ser26 cluster of TREX1 served to cap the nonscissile 5'-end of the DNA for precise removal of the short 3'-overhang in L- and Y-structural DNA or to wedge into the double-stranded region for further digestion along the duplex
Title: Structural basis for overhang excision and terminal unwinding of DNA duplexes by TREX1.
Trex1 is an upstream regulator of radiation-driven anti-tumor immunity. Trex1 induction may guide the selection of radiation dose and fractionation in patients treated with immunotherapy.
Title: DNA exonuclease Trex1 regulates radiotherapy-induced tumour immunogenicity.

Submit: New GeneRIF Correction See all GeneRIFs (28)

Variation

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Alleles

Alleles of this type are documented at Mouse Genome Informatics (MGI)

Endonuclease-mediated (14) 1 citation
Gene trapped (1)
Targeted (7) 1 citation

Interactions

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Products	Interactant	Other Gene	Complex	Source	Pubs	Description

General gene information

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Markers

RH131155 (e-PCR)
- UniSTS:214438

AU041952 (e-PCR)
- UniSTS:159920

NoName (e-PCR)
- UniSTS:236254

Readthrough Gm53191

Readthrough gene: Gm53191, Included gene: Atrip

Gene Ontology Provided by MGI

Function	Evidence Code	Pubs
enables 3'-5' exonuclease activity	IMP Inferred from Mutant Phenotype more info	PubMed
enables 3'-5' exonuclease activity	ISO Inferred from Sequence Orthology more info
enables 3'-5' exonuclease activity	ISS Inferred from Sequence or Structural Similarity more info
enables 3'-5'-DNA exonuclease activity	IBA Inferred from Biological aspect of Ancestor more info
enables 3'-5'-DNA exonuclease activity	IDA Inferred from Direct Assay more info	PubMed
enables 3'-5'-DNA exonuclease activity	ISO Inferred from Sequence Orthology more info
enables 3'-5'-DNA exonuclease activity	ISS Inferred from Sequence or Structural Similarity more info
enables DNA binding	IDA Inferred from Direct Assay more info	PubMed
enables DNA binding, bending	IDA Inferred from Direct Assay more info	PubMed
enables MutLalpha complex binding	ISO Inferred from Sequence Orthology more info	PubMed
enables MutSalpha complex binding	ISO Inferred from Sequence Orthology more info	PubMed
enables WW domain binding	IDA Inferred from Direct Assay more info	PubMed
enables adenyl deoxyribonucleotide binding	IDA Inferred from Direct Assay more info	PubMed
enables double-stranded DNA 3'-5' DNA exonuclease activity	IEA Inferred from Electronic Annotation more info
enables double-stranded DNA 3'-5' DNA exonuclease activity	ISO Inferred from Sequence Orthology more info
enables double-stranded DNA binding	IDA Inferred from Direct Assay more info	PubMed
enables identical protein binding	IPI Inferred from Physical Interaction more info	PubMed
enables magnesium ion binding	IDA Inferred from Direct Assay more info	PubMed
enables protein binding	IPI Inferred from Physical Interaction more info	PubMed
enables protein homodimerization activity	IPI Inferred from Physical Interaction more info	PubMed
enables single-stranded DNA binding	IDA Inferred from Direct Assay more info	PubMed

Process	Evidence Code	Pubs
acts_upstream_of_or_within CD86 biosynthetic process	IMP Inferred from Mutant Phenotype more info	PubMed
involved_in DNA catabolic process	IBA Inferred from Biological aspect of Ancestor more info
acts_upstream_of_or_within DNA catabolic process	IDA Inferred from Direct Assay more info	PubMed
acts_upstream_of_or_within DNA catabolic process	IMP Inferred from Mutant Phenotype more info	PubMed
acts_upstream_of_or_within DNA damage checkpoint signaling	IMP Inferred from Mutant Phenotype more info	PubMed
acts_upstream_of_or_within DNA damage response	IDA Inferred from Direct Assay more info	PubMed
acts_upstream_of_or_within DNA duplex unwinding	IDA Inferred from Direct Assay more info	PubMed
involved_in DNA metabolic process	IDA Inferred from Direct Assay more info	PubMed
acts_upstream_of_or_within DNA modification	IMP Inferred from Mutant Phenotype more info	PubMed
acts_upstream_of_or_within DNA replication	IMP Inferred from Mutant Phenotype more info	PubMed
acts_upstream_of_or_within DNA synthesis involved in UV-damage excision repair	IMP Inferred from Mutant Phenotype more info	PubMed
acts_upstream_of_or_within G2 phase	IMP Inferred from Mutant Phenotype more info	PubMed
acts_upstream_of_or_within T cell antigen processing and presentation	IMP Inferred from Mutant Phenotype more info	PubMed
acts_upstream_of_or_within activation of immune response	IMP Inferred from Mutant Phenotype more info	PubMed
acts_upstream_of_or_within adaptive immune response	IMP Inferred from Mutant Phenotype more info	PubMed
acts_upstream_of_or_within apoptotic cell clearance	IMP Inferred from Mutant Phenotype more info	PubMed
acts_upstream_of_or_within atrial cardiac muscle tissue development	IMP Inferred from Mutant Phenotype more info	PubMed
acts_upstream_of_or_within blood vessel development	IMP Inferred from Mutant Phenotype more info	PubMed
acts_upstream_of_or_within cellular response to UV	IDA Inferred from Direct Assay more info	PubMed
acts_upstream_of_or_within cellular response to gamma radiation	IMP Inferred from Mutant Phenotype more info	PubMed
acts_upstream_of_or_within cellular response to hydroxyurea	IMP Inferred from Mutant Phenotype more info	PubMed
acts_upstream_of_or_within cellular response to interferon-beta	IDA Inferred from Direct Assay more info	PubMed
acts_upstream_of_or_within cellular response to oxidative stress	IMP Inferred from Mutant Phenotype more info	PubMed
acts_upstream_of_or_within cellular response to reactive oxygen species	IMP Inferred from Mutant Phenotype more info	PubMed
acts_upstream_of_or_within cellular response to type I interferon	IMP Inferred from Mutant Phenotype more info	PubMed
acts_upstream_of_or_within defense response to virus	IMP Inferred from Mutant Phenotype more info	PubMed
acts_upstream_of_or_within determination of adult lifespan	IGI Inferred from Genetic Interaction more info	PubMed
acts_upstream_of_or_within determination of adult lifespan	IMP Inferred from Mutant Phenotype more info	PubMed
acts_upstream_of_or_within establishment of protein localization	IMP Inferred from Mutant Phenotype more info	PubMed
acts_upstream_of_or_within generation of precursor metabolites and energy	IMP Inferred from Mutant Phenotype more info	PubMed
acts_upstream_of_or_within heart morphogenesis	IMP Inferred from Mutant Phenotype more info	PubMed
acts_upstream_of_or_within heart process	IMP Inferred from Mutant Phenotype more info	PubMed
acts_upstream_of_or_within immune complex formation	IMP Inferred from Mutant Phenotype more info	PubMed
acts_upstream_of_or_within immune response	IMP Inferred from Mutant Phenotype more info	PubMed
acts_upstream_of_or_within immune response in brain or nervous system	IMP Inferred from Mutant Phenotype more info	PubMed
acts_upstream_of_or_within immunoglobulin production involved in immunoglobulin-mediated immune response	IMP Inferred from Mutant Phenotype more info	PubMed
acts_upstream_of_or_within inflammatory response	IMP Inferred from Mutant Phenotype more info	PubMed
acts_upstream_of_or_within inflammatory response to antigenic stimulus	IMP Inferred from Mutant Phenotype more info	PubMed
acts_upstream_of_or_within innate immune response	IMP Inferred from Mutant Phenotype more info	PubMed
acts_upstream_of_or_within interferon-alpha production	IMP Inferred from Mutant Phenotype more info	PubMed
acts_upstream_of_or_within kidney development	IMP Inferred from Mutant Phenotype more info	PubMed
acts_upstream_of_or_within lymphoid progenitor cell differentiation	IMP Inferred from Mutant Phenotype more info	PubMed
acts_upstream_of_or_within macrophage activation involved in immune response	IMP Inferred from Mutant Phenotype more info	PubMed
acts_upstream_of_or_within mitotic G1 DNA damage checkpoint signaling	IMP Inferred from Mutant Phenotype more info	PubMed
involved_in negative regulation of cGAS/STING signaling pathway	IEA Inferred from Electronic Annotation more info
involved_in negative regulation of cGAS/STING signaling pathway	ISO Inferred from Sequence Orthology more info
involved_in negative regulation of innate immune response	ISO Inferred from Sequence Orthology more info
involved_in negative regulation of innate immune response	ISS Inferred from Sequence or Structural Similarity more info
acts_upstream_of_or_within negative regulation of type I interferon-mediated signaling pathway	IMP Inferred from Mutant Phenotype more info	PubMed
acts_upstream_of_or_within organ or tissue specific immune response	IMP Inferred from Mutant Phenotype more info	PubMed
acts_upstream_of_or_within protein stabilization	IDA Inferred from Direct Assay more info	PubMed
acts_upstream_of_or_within regulation of T cell activation	IGI Inferred from Genetic Interaction more info	PubMed
acts_upstream_of_or_within regulation of catalytic activity	IMP Inferred from Mutant Phenotype more info	PubMed
acts_upstream_of_or_within regulation of cellular respiration	IMP Inferred from Mutant Phenotype more info	PubMed
acts_upstream_of_or_within regulation of fatty acid metabolic process	IMP Inferred from Mutant Phenotype more info	PubMed
acts_upstream_of_or_within regulation of gene expression	IGI Inferred from Genetic Interaction more info	PubMed
acts_upstream_of_or_within regulation of gene expression	IMP Inferred from Mutant Phenotype more info	PubMed
acts_upstream_of_or_within regulation of glycolytic process	IMP Inferred from Mutant Phenotype more info	PubMed
acts_upstream_of_or_within regulation of immunoglobulin production	IGI Inferred from Genetic Interaction more info	PubMed
acts_upstream_of_or_within regulation of immunoglobulin production	IMP Inferred from Mutant Phenotype more info	PubMed
acts_upstream_of_or_within regulation of inflammatory response	IGI Inferred from Genetic Interaction more info	PubMed
acts_upstream_of_or_within regulation of innate immune response	IMP Inferred from Mutant Phenotype more info	PubMed
acts_upstream_of_or_within regulation of lipid biosynthetic process	IGI Inferred from Genetic Interaction more info	PubMed
acts_upstream_of_or_within regulation of lysosome organization	IMP Inferred from Mutant Phenotype more info	PubMed
acts_upstream_of_or_within regulation of metabolic process	IGI Inferred from Genetic Interaction more info	PubMed
acts_upstream_of_or_within regulation of metabolic process	IMP Inferred from Mutant Phenotype more info	PubMed
acts_upstream_of_or_within regulation of protein complex stability	IMP Inferred from Mutant Phenotype more info	PubMed
acts_upstream_of_or_within regulation of tumor necrosis factor production	IMP Inferred from Mutant Phenotype more info	PubMed
acts_upstream_of_or_within regulation of type I interferon production	IGI Inferred from Genetic Interaction more info	PubMed
acts_upstream_of_or_within regulation of type I interferon production	IMP Inferred from Mutant Phenotype more info	PubMed
acts_upstream_of_or_within response to UV	IMP Inferred from Mutant Phenotype more info	PubMed
acts_upstream_of_or_within retrotransposition	IMP Inferred from Mutant Phenotype more info	PubMed
acts_upstream_of_or_within type I interferon-mediated signaling pathway	IMP Inferred from Mutant Phenotype more info	PubMed

Component	Evidence Code	Pubs
is_active_in cytoplasm	IBA Inferred from Biological aspect of Ancestor more info
located_in cytoplasm	IDA Inferred from Direct Assay more info	PubMed
located_in cytosol	IDA Inferred from Direct Assay more info	PubMed
located_in cytosol	ISO Inferred from Sequence Orthology more info
located_in endoplasmic reticulum	IDA Inferred from Direct Assay more info	PubMed
located_in endoplasmic reticulum membrane	ISO Inferred from Sequence Orthology more info
located_in nuclear replication fork	IDA Inferred from Direct Assay more info	PubMed
located_in nucleus	IDA Inferred from Direct Assay more info	PubMed
part_of oligosaccharyltransferase complex	IDA Inferred from Direct Assay more info	PubMed
part_of protein-DNA complex	IDA Inferred from Direct Assay more info	PubMed

General protein information

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Preferred Names: three-prime repair exonuclease 1

Names: 3'-5' exonuclease TREX1; DNase III; three prime exonuclease 1; trophoblast expressed 1

NP_001012236.1

EC 3.1.11.2

NP_035767.4

EC 3.1.11.2

NCBI Reference Sequences (RefSeq)

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RefSeqs maintained independently of Annotated Genomes

These reference sequences exist independently of genome builds. Explain

These reference sequences are curated independently of the genome annotation cycle, so their versions may not match the RefSeq versions in the current genome build. Identify version mismatches by comparing the version of the RefSeq in this section to the one reported in Genomic regions, transcripts, and products above.

mRNA and Protein(s)

NM_001012236.1 → NP_001012236.1 three-prime repair exonuclease 1

See identical proteins and their annotated locations for NP_001012236.1

Status: VALIDATED

Description

Transcript Variant: This variant (2) differs in the 5' UTR compared to variant 1. Variants 1 and 2 encode the same isoform.

Source sequence(s)

AK087832, AU041952

Consensus CDS

CCDS23544.1

UniProtKB/Swiss-Prot

Q3TAD7, Q91XB0, Q9D6W2, Q9R1B0

Related

ENSMUSP00000107684.2, ENSMUST00000112053.2

Conserved Domains (2) summary

cd06136
Location:14 → 211

TREX1_2; DEDDh 3'-5' exonuclease domain of three prime repair exonuclease (TREX)1, TREX2, and similar proteins

COG0847
Location:13 → 204

DnaQ; DNA polymerase III, epsilon subunit or related 3'-5' exonuclease [Replication, recombination and repair]
NM_011637.6 → NP_035767.4 three-prime repair exonuclease 1

See identical proteins and their annotated locations for NP_035767.4

Status: VALIDATED

Description

Transcript Variant: This variant (1) represents the longer transcript.

Source sequence(s)

AU041952, BY234700, CA323364, CO041929

Consensus CDS

CCDS23544.1

UniProtKB/Swiss-Prot

Q3TAD7, Q91XB0, Q9D6W2, Q9R1B0

Related

ENSMUSP00000050971.5, ENSMUST00000061973.5

Conserved Domains (2) summary

cd06136
Location:14 → 211

TREX1_2; DEDDh 3'-5' exonuclease domain of three prime repair exonuclease (TREX)1, TREX2, and similar proteins

COG0847
Location:13 → 204

DnaQ; DNA polymerase III, epsilon subunit or related 3'-5' exonuclease [Replication, recombination and repair]