REPAIRtoire - a database of DNA repair pathways

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TREX1

three prime repair exonuclease 1

On chromosome: 3p21.31
Known also as: CRV; AGS1; DRN3; HERNS; DKFZp434J0310;


NCBI Gene ID: 11277
NCBI Ensembl Id: ENSG00000213689
MIM Id: (from NCBI OMIM database) 606609
Species: Homo sapiens

This gene encodes the major 3'->5' DNA exonuclease in human cells. The protein is a non-processive exonuclease that may serve a proofreading function for a human DNA polymerase. It is also a component of the SET complex, and acts to rapidly degrade 3' ends of nicked DNA during granzyme A-mediated cell death. Mutations in this gene result in Aicardi-Goutieres syndrome, chilblain lupus, and Cree encephalitis. Multiple transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq]


Gene sequence:
[Download sequence]

Proteins coded by this gene:
References:

Authors Title Journal
Hoss M, Robins P, Naven TJ, Pappin DJ, Sgouros J, Lindahl T A human DNA editing enzyme homologous to the Escherichia coli DnaQ/MutD protein. EMBO J July 1, 1999
Mazur DJ, Perrino FW Identification and expression of the TREX1 and TREX2 cDNA sequences encoding mammalian 3'-->5' exonucleases. J Biol Chem July 9, 1999
Mazur DJ, Perrino FW Structure and expression of the TREX1 and TREX2 3' --> 5' exonuclease genes. J Biol Chem May 4, 2001
Yoshioka K, Yoshioka Y, Hsieh P ATR kinase activation mediated by MutSalpha and MutLalpha in response to cytotoxic O6-methylguanine adducts. Mol Cell May 19, 2006

Last modification of this entry: Oct. 6, 2010.

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