Product Details

Purity

Greater than 85% as determined by SDS-PAGE.

Target Names

TREX1

Uniprot No.

Q9NSU2

Research Area

Epigenetics and Nuclear Signaling

Alternative Names

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

Species

Homo sapiens (Human)

Source

E.coli

Expression Region

1-242aa

Target Protein Sequence

MGPGARRQGRIVQGRPEMCFCPPPTPLPPLRILTLGTHTPTPCSSPGSAAGTYPTMGSQALPPGPMQTLIFFDMEATGLPFSQPKVTELCLLAVHRCALESPPTSQGPPPTVPPPPRVVDKLSLCVAPGKACSPAASEITGLSTAVLAAHGRQCFDDNLANLLLAFLRRQPQPWCLVAHNGDRYDFPLLQAELAMLGLTSALDGAFCVDSITALKALERASSPSEHGPRKSYSLGSIYTRLY
Note: The complete sequence may include tag sequence, target protein sequence, linker sequence and extra sequence that is translated with the protein sequence for the purpose(s) of secretion, stability, solubility, etc.
If the exact amino acid sequence of this recombinant protein is critical to your application, please explicitly request the full and complete sequence of this protein before ordering.

Mol. Weight

32.7 kDa

Protein Length

Partial

Tag Info

N-terminal 10xHis-tagged and C-terminal Myc-tagged

Form

Liquid or Lyophilized powder
Note: We will preferentially ship the format that we have in stock, however, if you have any special requirement for the format, please remark your requirement when placing the order, we will prepare according to your demand.

Buffer

If the delivery form is liquid, the default storage buffer is Tris/PBS-based buffer, 5%-50% glycerol.
Note: If you have any special requirement for the glycerol content, please remark when you place the order.
If the delivery form is lyophilized powder, the buffer before lyophilization is Tris/PBS-based buffer, 6% Trehalose.

Reconstitution

We recommend that this vial be briefly centrifuged prior to opening to bring the contents to the bottom. Please reconstitute protein in deionized sterile water to a concentration of 0.1-1.0 mg/mL.We recommend to add 5-50% of glycerol (final concentration) and aliquot for long-term storage at -20°C/-80°C. Our default final concentration of glycerol is 50%. Customers could use it as reference.

Troubleshooting and FAQs

Protein FAQs

Storage Condition

Store at -20°C/-80°C upon receipt, aliquoting is necessary for mutiple use. Avoid repeated freeze-thaw cycles.

Shelf Life

The shelf life is related to many factors, storage state, buffer ingredients, storage temperature and the stability of the protein itself.
Generally, the shelf life of liquid form is 6 months at -20°C/-80°C. The shelf life of lyophilized form is 12 months at -20°C/-80°C.

Lead Time

Delivery time may differ from different purchasing way or location, please kindly consult your local distributors for specific delivery time.

Notes

Repeated freezing and thawing is not recommended. Store working aliquots at 4°C for up to one week.

Datasheet & COA

Please contact us to get it.

Description

The gene fragment corresponding to the 1-242aa of the human TREX1 protein was synthesized, with appropriate restriction sites suitable for in-frame cloning into an expression vector, with N-terminal 10xHis tag and C-terminal Myc tag. The E.coli was transformed with the expression vector, and the clone was expressed upon certain induction. After the induced cell centrifugation, the recombinant protein was purified from the cell extract and presented as N-terminal 10xHis-tagged and C-terminal Myc-tagged fusion. This recombinant human TREX1 protein's purity is greater than 85% assayed by SDS-PAGE. The TREX1 protein ran to a band of about 33 kDa molecular weight on the gel, indicating a glycosylated form of the protein.

TREX1 is a member of the DEDD family of 3’ → 5’, whose members are defined by a conserved Asp-Glu-Asp-Asp motif that facilitates catalytic activity. Members of the DEDD nuclease family frequently have a role in DNA replication and/or repair. More than sixty TREX1 mutations have now been identified that exhibit dominant and recessive genetics and occur as inherited or de novo mutations, dependent upon the specific mutant allele. TREX1 disease alleles include missense mutations, insertions, duplications, and frame shifts that locate to positions throughout the 314-amino acid-coding gene. There is a causal relationship between TREX1 genetic variants and multiple mechanisms of TREX1 enzyme dysfunction that have now been linked to a spectrum of autoimmune diseases in human. Most of the TREX1 mutations affecting the catalytic domain are recessive and are largely associated with Aicardi-Goutières Syndrome (AGS) or Familial Chilblains Lupus (FCL).

Target Background

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.

Gene References into Functions

data identifies miR-103 regulation of TREX1 as a potent modulator of the tumour microenvironment. PMID: 27886180
Thus, the intracellular level of TREX1 pivotally modulates innate immune induction by HIV-1. Partial HIV-1 genomes are the TREX1 target and are sensed by cGAS. PMID: 29769349
TREX1-deficient neurons also exhibited increased apoptosis and formed three-dimensional cortical organoids of reduced size. PMID: 28803918
Homozygote R114C mutation in TREX1 gene was shown in two siblings, one with chilblains and another with chilblains accompanied with cerebral vasculitis. PMID: 28919362
TREX1 frame-shift mutations induce autoantibodies against mostly non-nuclear antigens, patients carrying these mutations are likely be ANA negative PMID: 28325644
The activation of interferon-induced genes is controlled by the JAK-STAT system; therefore, JAK inhibitors were successfully used in several cases to treat type 1 interferonopathies. Experience with this treatment modality is continuously growing. PMID: 28389709
TheTREX1 and STING, which are opposing regulators of the cytosolic DNA-sensing pathway. PMID: 28475463
the cell-cycle-dependent post-translation modification of TREX1 regulates its interaction with OST. PMID: 28297665
The effect of topical TREX1 knockdown and local interferon production on HIV transmission in human cervicovaginal explants and humanized mice, is reported. PMID: 27184854
data do not support the concept of retroelement-derived cDNA as key triggers of systemic autoimmunity in Trex1-deficient humans and mice PMID: 28835460
Aicardi-Goutieres syndrome protein TREX1 suppresses L1 and maintains genome integrity through exonuclease-independent ORF1p depletion. PMID: 28334850
Analysis of mRNA expression profiles in breast tumors demonstrates that those with lower Trex1 and higher BLM and EXO1 expression levels are associated with poor prognosis PMID: 28279982
showed that patients with SSc or SLE have AAb against EphB2, a protein involved in angiogenesis, and THEX1, a 3'-5' exoribonuclease involved in histone mRNA degradation. PMID: 27617966
The expression of TREX1 was closely related to the cytobiology characteristics of osteosarcoma stem cell. PMID: 27881153
Heterozygous mutations in TREX1 were reported in SLE patients. PMID: 26150267
synonymous variant p.(Tyr177) associated with an increased risk of systemic scleroderma; association strongest in patients with limited cutaneous scleroderma, in particular in those with more severe digital vascular disease PMID: 27574969
This study reviewed that Neurologic Phenotypes Associated with Mutations in TREX1 in patients with Aicardi-Goutieres Syndrome. PMID: 27643693
Aicardie-Goutieres syndrome is described in a patient with a homozygous p.Arg114His mutation in the TREX1 gene. PMID: 28089741
CDK11 was found associated with the TREX/THOC, which recruited this kinase to DNA. Once at the viral genome, CDK11 phosphorylated serines at position 2 in the CTD of RNAPII, which increased levels of cleavage and polyadenylation factors at the HIV 3' end. In its absence, cleavage of viral transcripts was greatly attenuated. PMID: 26567509
Aicardi-Goutieres syndrome 1 is caused by mutations in the three prime repair exonuclease 1 gene (TREX1, MIM 606609). PMID: 25906927
Functional analysis of retinal vasculopathy with cerebral leukodystrophy-associated TREX1 T270 frameshift-mutated fibroblasts showed a prevalent localization of the protein in the cytosol, rather than in the perinuclear region PMID: 25213617
Here we briefly describe the human diseases so-far associated with mutations in TREX1, which include Aicardi-Goutieres syndrome, familial chilblain lupus, systemic lupus erythematosus and retinal vasculopathy with cerebral leukodystrophy PMID: 25731743
Studies indicate that the biosensing strategy is based on the protection of DNA duplex from exonuclease III (Exo III)-mediated digestion by specific binding of estrogen receptor (ER) to its DNA response element. PMID: 23681011
The authors observed that the minor allele of SNP rs3135941 in Trex1 is associated with faster HIV-1 disease progression. PMID: 25162766
Unlike previously reported TREX1 mutations, which affect Exo I or Exo II domains, the mutation presented here alters the Exo III domain, suggesting a role of mutations within the catalytic Exo domains in the pathogenesis of familial chilblain lupus PMID: 25517357
Data suggest that the 3'-5' DNA exonuclease TREX1 may contribute to stabilization of poly(ADP-ribose) polymerase-1 (PARP1) levels in the DNA damage response and its activity. PMID: 25278026
knocking out the DNA sensor cyclic GMP-AMP synthase completely abrogates spontaneous induction of IFN-stimulated genes in TREX1-deficient cells. PMID: 24813208
Data indicate that the single nucleotide polymorphism rs3135945 was significantly associated with HIV infection, emphasizing the involvement of TREX-1 in the anti-HIV response. PMID: 23773365
This study contributes to the demonstration that TREX1 is involved in autoimmune diseases and proposes that the spectrum of involved autoimmune diseases can be broader and includes systemic sclerosis . PMID: 24224166
Spontaneous type I INF dependent cutaneous pathology in TREX1 deficiency illustrates common pathogenetic pathway in chilblain lupus. PMID: 24270665
These data provide compelling evidence for the required TREX1 dimeric structure for full catalytic function. PMID: 24616097
A nationwide survey of Aicardi-Goutieres syndrome patients identifies a strong association between dominant TREX1 mutations and chilblain lesions in Japan. PMID: 24300241
Screening of our patient cohort yielded heterozygous TREX1 mutations in two patients with early-onset cerebrovascular disease PMID: 23881107
TREX1 mutation is not a common cause of hereditary small vessel diseases of the brain PMID: 23602593
TREX1 post-translational modification indicates an additional mechanism by which mutations disrupt TREX1 biology, leading to human autoimmune disease. PMID: 23979357
TREX1 gene variants confer an increased risk for the development of systemic lupus erythematosus. PMID: 24034389
TREX1 knockdown resulted in enhanced cell death following nimustine. PMID: 23578789
Aicardi-Goutieres syndrome and familial chilblain lupus can be caused by a heterozygous TREX1 p.Asp18Asn mutation. PMID: 22829693
Despite its central role in the HIV-1 infection process, genetic diversity at TREX1 is not a major determinant of susceptibility to infection in humans. PMID: 22526516
The structures of the mutant TREX1 proteins provide insight into the dysfunction relating to human disease. PMID: 22071149
TREX1 residues in one protomer contributing to DNA degradation catalyzed in the opposing protomer and help to explain the dimeric TREX1 structure required for full catalytic competency. PMID: 21937424
failure to degrade genomic dsDNA is a principal pathway of immune activation in TREX1-mediated autoimmune disease. PMID: 21808053
data confirm and expand previous reports and provide additional support for the involvement of TREX1 in lupus pathogenesis PMID: 21270825
A single case of a heterozygous TREX1 mutation has been associated with Aicardi-Goutieres syndrome, an encephalopathy often resembling congenital infection. PMID: 20799324
TREX1 bound to cytosolic HIV DNA and digested excess HIV DNA that would otherwise activate interferon expression via a pathway dependent on the kinase TBK1, the adaptor STING and the transcription factor IRF3 PMID: 20871604
The deficiency of Trex-1 in rheumatoid arthritis synovial fibroblasts allows a longer half-life of gene products encoded by active endogenous L1 retrotransposons. PMID: 20496420
TREX1 mutations do not play a role in the pathogenesis of Sporadic inclusion body myositis. PMID: 20192983
TREX1 may act in degrading DNA in all cell types undergoing a dying process before phagocytosis occurs. PMID: 19617005
TREX1, encoding the major mammalian 3' --> 5' DNA exonuclease, is the AGS1 gene, and AGS-causing mutations result in abrogation of TREX1 enzyme activity. PMID: 16845398
Heterozygous mutations in TREX1 cause familial chilblain lupus and dominant Aicardi-Goutieres syndrome. PMID: 17357087

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Involvement in disease

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

Subcellular Location

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

Protein Families

Exonuclease superfamily, TREX family

Tissue Specificity

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

Database Links

HGNC: 12269

OMIM: 152700

KEGG: hsa:11277

STRING: 9606.ENSP00000390478

UniGene: Hs.707026

Code	CSB-EP865133HU1
Abbreviation	Recombinant Human TREX1 protein, partial
MSDS	MSDS Datasheet
Size	US$306
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Image	(Tris-Glycine gel) Discontinuous SDS-PAGE (reduced) with 5% enrichment gel and 15% separation gel.
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Recombinant Human Three-prime repair exonuclease 1 (TREX1), partial

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