Mouse Anti-TREX1 Recombinant Antibody (CBYJT-4633) (CBMAB-T4163-YJ)

Mouse

Human

CBYJT-4633

IgG1

WB, ICC, IF, IP

The exact sequence is proprietary

Human

IgG1

Monoclonal

The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

PBS, pH 7.4

Store at 4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

TREX1 Gene(Protein Coding) Three Prime Repair Exonuclease 1

TREX1 is a nuclear protein with 3' exonuclease activity. TREX1 may function in DNA repair and serve as a proofreading function for DNA polymerase. Mutations in TREX1 result in Aicardi-Goutieres syndrome, chilblain lupus, Cree encephalitis, and other diseases of the immune system.

Three Prime Repair Exonuclease 1; Deoxyribonuclease III; 3-5 Exonuclease TREX1; DNase III; Three-Prime Repair Exonuclease 1; Aicardi-Goutieres Syndrome 1; 3 Repair Exonuclease 1

Major cellular 3'-to-5' DNA exonuclease which digests single-stranded DNA (ssDNA) and double-stranded DNA (dsDNA) with mismatched 3' termini (PubMed:10391904, PubMed:10393201, PubMed:17293595).
Prevents cell-intrinsic initiation of autoimmunity (PubMed:10391904, PubMed:10393201, PubMed:17293595).
Acts by metabolizing DNA fragments from endogenous retroelements, including L1, LTR and SINE elements (PubMed:10391904, PubMed:10393201, PubMed:17293595).
Plays a key role in degradation of DNA fragments at cytosolic micronuclei arising from genome instability: its association with the endoplasmic reticulum membrane directs TREX1 to ruptured micronuclei, leading to micronuclear DNA degradation (PubMed:33476576).
Micronuclear DNA degradation is required to limit CGAS activation and subsequent inflammation (PubMed:33476576).
Unless degraded, these DNA fragments accumulate in the cytosol and activate the cGAS-STING innate immune signaling, leading to the production of type I interferon (PubMed:33476576).
Prevents chronic ATM-dependent checkpoint activation, by processing ssDNA polynucleotide species arising from the processing of aberrant DNA replication intermediates (PubMed:18045533).
Inefficiently degrades oxidized DNA, such as that generated upon antimicrobial reactive oxygen production or upon absorption of UV light (PubMed:23993650).
During GZMA-mediated cell death, contributes to DNA damage in concert with NME1 (PubMed:16818237).
NME1 nicks one strand of DNA and TREX1 removes bases from the free 3' end to enhance DNA damage and prevent DNA end reannealing and rapid repair (PubMed:16818237).

Biological Process activation of immune response Source:Ensembl
Biological Process apoptotic cell clearance Source:Ensembl
Biological Process atrial cardiac muscle tissue development Source:Ensembl
Biological Process blood vessel development Source:Ensembl
Biological Process CD86 biosynthetic process Source:Ensembl
Biological Process cellular response to gamma radiation Source:Ensembl
Biological Process cellular response to hydroxyurea Source:Ensembl
Biological Process cellular response to interferon-beta Source:Ensembl
Biological Process cellular response to reactive oxygen species Source:Ensembl
Biological Process defense response to virus Source:Ensembl
Biological Process determination of adult lifespan Source:Ensembl
Biological Process DNA catabolic process, exonucleolytic Source:GO_Central1 Publication
Biological Process DNA duplex unwinding Source:Ensembl
Biological Process DNA metabolic process Source:UniProtKB
Biological Process DNA modification Source:Ensembl
Biological Process DNA recombination Source:UniProtKB1 Publication
Biological Process DNA repair Source:ProtInc1 Publication
Biological Process DNA replication Source:UniProtKB1 Publication
Biological Process DNA synthesis involved in UV-damage excision repair Source:Ensembl
Biological Process establishment of protein localization Source:Ensembl
Biological Process generation of precursor metabolites and energy Source:Ensembl
Biological Process heart morphogenesis Source:Ensembl
Biological Process heart process Source:Ensembl
Biological Process immune complex formation Source:Ensembl
Biological Process immune response in brain or nervous system Source:Ensembl
Biological Process inflammatory response to antigenic stimulus Source:Ensembl
Biological Process kidney development Source:Ensembl
Biological Process lymphoid progenitor cell differentiation Source:Ensembl
Biological Process macrophage activation involved in immune response Source:Ensembl
Biological Process mismatch repair Source:UniProtKB1 Publication
Biological Process mitotic G1 DNA damage checkpoint signaling Source:Ensembl
Biological Process negative regulation of innate immune response Source:UniProtKB1 Publication
Biological Process negative regulation of type I interferon-mediated signaling pathway Source:Ensembl
Biological Process protein stabilization Source:Ensembl
Biological Process regulation of catalytic activity Source:Ensembl
Biological Process regulation of cellular respiration Source:Ensembl
Biological Process regulation of fatty acid metabolic process Source:Ensembl
Biological Process regulation of glycolytic process Source:Ensembl
Biological Process regulation of immunoglobulin production Source:Ensembl
Biological Process regulation of inflammatory response Source:Ensembl
Biological Process regulation of lipid biosynthetic process Source:Ensembl
Biological Process regulation of lysosome organization Source:Ensembl
Biological Process regulation of protein complex stability Source:Ensembl
Biological Process regulation of T cell activation Source:Ensembl
Biological Process regulation of tumor necrosis factor production Source:Ensembl
Biological Process regulation of type I interferon production Source:Ensembl
Biological Process T cell antigen processing and presentation Source:Ensembl
Biological Process transposition, RNA-mediated Source:Ensembl
Biological Process type I interferon signaling pathway Source:Ensembl

Nucleus
Cytoplasm, cytosol
Endoplasmic reticulum membrane
Retained in the cytoplasm through the C-terminal region (By similarity).
Localization to the endoplasmic reticulum membrane is required to direct TREX1 to ruptured micronuclei (PubMed:33476576).
In response to DNA damage, translocates to the nucleus where it is specifically recruited to replication foci (PubMed:16818237).
Translocation to the nucleus also occurs during GZMA-mediated cell death (PubMed:16818237).

Aicardi-Goutieres syndrome 1 (AGS1):
A form of Aicardi-Goutieres syndrome, a genetically heterogeneous disease characterized by cerebral atrophy, leukoencephalopathy, intracranial calcifications, chronic cerebrospinal fluid (CSF) lymphocytosis, increased CSF alpha-interferon, and negative serologic investigations for common prenatal infection. Clinical features as thrombocytopenia, hepatosplenomegaly and elevated hepatic transaminases along with intermittent fever may erroneously suggest an infective process. Severe neurological dysfunctions manifest in infancy as progressive microcephaly, spasticity, dystonic posturing and profound psychomotor retardation. Death often occurs in early childhood.
Systemic lupus erythematosus (SLE):
A chronic, relapsing, inflammatory, and often febrile multisystemic disorder of connective tissue, characterized principally by involvement of the skin, joints, kidneys and serosal membranes. It is of unknown etiology, but is thought to represent a failure of the regulatory mechanisms of the autoimmune system. The disease is marked by a wide range of system dysfunctions, an elevated erythrocyte sedimentation rate, and the formation of LE cells in the blood or bone marrow.
Chilblain lupus 1 (CHBL1):
A rare cutaneous form of lupus erythematosus. Affected individuals present with painful bluish-red papular or nodular lesions of the skin in acral locations precipitated by cold and wet exposure.
Vasculopathy, retinal, with cerebral leukoencephalopathy and systemic manifestations (RVCLS):
An adult-onset, autosomal dominant endotheliopathy affecting the microvessels of the brain. It results in central nervous system degeneration and retinopathy, with progressive loss of vision, stroke, motor impairment, and cognitive decline. The ocular manifestations are characterized by telangiectasias, microaneurysms and retinal capillary obliteration starting in the macula. Diseased cerebral white matter has prominent small infarcts that often coalesce to pseudotumors. A subset of patients have systemic vascular involvement that can manifest as Raynaud phenomenon, micronodular cirrhosis, and glomerular dysfunction.

Ubiquitinated, but not targeted to proteasomal degradation. Ubiquitination may be important for interaction with UBQLN1.