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Article

Aicardi-Goutières syndrome protein TREX1 suppresses L1 and maintains genome integrity through exonuclease-independent ORF1p depletion.

Nucleic acids research - 5 May 2017

Li Peng, Du Juan, Goodier John L, Hou Jingwei, Kang Jian, Kazazian Haig H, Zhao Ke, Yu Xiao-Fang

Abstract excerpt

Maintaining genome integrity is important for cells and damaged DNA triggers autoimmunity. Previous studies have reported that Three-prime repair exonuclease 1(TREX1), an endogenous DNA exonuclease, prevents immune activation by depleting damaged DNA, thus preventing the development of certain autoimmune diseases. Consistently, mutations in TREX1 are linked with autoimmune diseases such as systemic lupus...

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Source

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Identifiers

PMID: 28334850
DOI: 10.1093/nar/gkx178
PMCID: PMC5416883

Genes

TREX1

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 4 Sept 2026.

Related research

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Aicardi-Goutières syndrome protein TREX1 suppresses L1 and maintains genome integrity through exonuclease-independent ORF1p depletion.PMID 28334850Read ↗

TREX1 exonuclease in immunity and diseaseSemantic proximity Read ↗

The TREX1 double-stranded DNA degradation activity is defective in dominant mutations associated with autoimmune disease.Semantic proximity Read ↗

Exonuclease TREX1 degrades double-stranded DNA to prevent spontaneous lupus-like inflammatory disease.Semantic proximity Read ↗

Trex1 Exonuclease Degrades ssDNA to Prevent Chronic Checkpoint Activation and Autoimmune DiseaseSemantic proximity Read ↗

TREX1 - Apex predator of cytosolic DNA metabolism.Semantic proximity Read ↗

Mutations in the gene encoding the 3'-5' DNA exonuclease TREX1 cause Aicardi-Goutières syndrome at the AGS1 locus.Semantic proximity Read ↗

Defects in DNA degradation revealed in crystal structures of TREX1 exonuclease mutations linked to autoimmune disease.Semantic proximity Read ↗

Heterozygous mutations in TREX1 cause familial chilblain lupus and dominant Aicardi-Goutieres syndrome.Semantic proximity Read ↗

A mutation in TREX1 that impairs susceptibility to granzyme A-mediated cell death underlies familial chilblain lupus.Semantic proximity Read ↗

The 3'-5' DNA exonuclease TREX1 directly interacts with poly(ADP-ribose) polymerase-1 (PARP1) during the DNA damage response.Semantic proximity Read ↗

Mutations in the non-catalytic polyproline motif destabilize TREX1 and amplify cGAS-STING signalingSemantic proximity Read ↗

Trex1 regulates lysosomal biogenesis and interferon-independent activation of antiviral genes.Semantic proximity Read ↗

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