Rabbit anti-Human TREX1 Polyclonal Antibody

Uniprot Id

Q9NSU2

Target Species

Human

Target Name

TREX1

Target Full Name

Three-prime repair exonuclease 1

Target Function

Major cellular 3'-to-5' DNA exonuclease which digests single-stranded DNA (ssDNA) and double-stranded DNA (dsDNA) with mismatched 3' termini. Prevents cell-intrinsic initiation of autoimmunity. Acts by metabolizing DNA fragments from endogenous retroelements, including L1, LTR and SINE elements. Unless degraded, these DNA fragments accumulate in the cytosol and activate the IFN-stimulatory DNA (ISD) response and innate immune signaling. Prevents chronic ATM-dependent checkpoint activation, by processing ssDNA polynucleotide species arising from the processing of aberrant DNA replication intermediates. Inefficiently degrades oxidized DNA, such as that generated upon antimicrobial reactive oxygen production or upon absorption of UV light. During GZMA-mediated cell death, contributes to DNA damage in concert with NME1. 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.

Target Involvement

Aicardi-Goutieres syndrome 1 (AGS1); Systemic lupus erythematosus (SLE); Chilblain lupus 1 (CHBL1); Vasculopathy, retinal, with cerebral leukodystrophy (RVCL)

Target Subcellular Location

Nucleus. Cytoplasm, cytosol. Endoplasmic reticulum membrane; Peripheral membrane protein.

Target Protein Families

Exonuclease superfamily, TREX family

Target Tissue Specificity

Detected in thymus, spleen, liver, brain, heart, small intestine and colon.

Target Research Area

Epigenetics and Nuclear Signaling

Target Synonyms

TREX1; Three-prime repair exonuclease 1; EC 3.1.11.2; 3'-5' exonuclease TREX1; Deoxyribonuclease III; DNase III

Target Background

This gene encodes a nuclear protein with 3' exonuclease activity. The encoded protein may play a role in DNA repair and serve as a proofreading function for DNA polymerase. Mutations in this gene result in Aicardi-Goutieres syndrome, chilblain lupus, Cree encephalitis, and other diseases of the immune system. Alternative splicing results in multiple transcript variants.