DCLRE1C Antibody
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中文名稱：DCLRE1C兔多克隆抗體
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貨號：CSB-PA050038
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規(guī)格：￥1090
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其他：
產(chǎn)品詳情
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Uniprot No.：
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基因名：DCLRE1C
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別名：A SCID antibody; A SCID protein antibody; Artemis protein antibody; ASCID antibody; DCLRE1C antibody; DCLRE1C DNA cross link repair 1C antibody; DCLRE1C protein antibody; DCLREC1C antibody; DCR1C_HUMAN antibody; DNA cross link repair 1C antibody; DNA cross link repair 1C protein antibody; DNA cross-link repair 1C protein antibody; FLJ11360 antibody; FLJ36438 antibody; hSNM1C antibody; OTTHUMP00000045150 antibody; Protein A-SCID antibody; Protein ARTEMIS antibody; PSO2 homolog antibody; RS SCID antibody; SCIDA antibody; Severe combined immunodeficiency type a antibody; SNM1 homolog C antibody; SNM1 like protein antibody; SNM1-like protein antibody; SNM1C antibody
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宿主：Rabbit
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反應種屬：Human
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免疫原：Synthesized peptide derived from Human Artemis around the non-phosphorylation site of S516.
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免疫原種屬：Homo sapiens (Human)
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標記方式：Non-conjugated
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抗體亞型：IgG
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純化方式：The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
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濃度：It differs from different batches. Please contact us to confirm it.
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保存緩沖液：Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
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產(chǎn)品提供形式：Liquid
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應用范圍：IHC, IF, ELISA
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推薦稀釋比：
Application Recommended Dilution IHC 1:100-1:300 IF 1:200-1:1000 ELISA 1:10000 -
Protocols：
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儲存條件：Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
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貨期：Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
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用途：For Research Use Only. Not for use in diagnostic or therapeutic procedures.
產(chǎn)品評價
相關產(chǎn)品
靶點詳情
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功能：Nuclease involved in DNA non-homologous end joining (NHEJ); required for double-strand break repair and V(D)J recombination. Required for V(D)J recombination, the process by which exons encoding the antigen-binding domains of immunoglobulins and T-cell receptor proteins are assembled from individual V, (D), and J gene segments. V(D)J recombination is initiated by the lymphoid specific RAG endonuclease complex, which generates site specific DNA double strand breaks (DSBs). These DSBs present two types of DNA end structures: hairpin sealed coding ends and phosphorylated blunt signal ends. These ends are independently repaired by the non homologous end joining (NHEJ) pathway to form coding and signal joints respectively. This protein exhibits single-strand specific 5'-3' exonuclease activity in isolation and acquires endonucleolytic activity on 5' and 3' hairpins and overhangs when in a complex with PRKDC. The latter activity is required specifically for the resolution of closed hairpins prior to the formation of the coding joint. Also required for the repair of complex DSBs induced by ionizing radiation, which require substantial end-processing prior to religation by NHEJ.
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基因功能參考文獻：
- Data suggest that stimulation of Artemis nuclease/DCLRE1C activity by XRCC4-DNA ligase IV hetero-complex and efficiency of blunt-end ligation are determined by structural configurations at the DNA ends. (XRCC4 = X-ray repair cross complementing 4) PMID: 28696258
- An N-terminal fragment comprising the catalytic domain can interact both with itself and with a C-terminal fragment. Amino acid exchanges N456A+S457A+E458Q in the C terminus of full-length SCIDA resulted in unmasking of the N terminus and in increased SCIDA activity in cellular V(D)J recombination assays. PMID: 28082683
- Data demonstrate that DCLRE1C mutations can cause a phenotype presenting as only antibody deficiency. PMID: 26476407
- DCLRE1C and NCF1 mutations have been found by whole-genome sequencing to cause primary immunodeficiency in unrelated patients. PMID: 25981738
- the nature and location of mutations correlate with the clinical phenotype of severe combined immunodeficiency PMID: 25917813
- uncovered a nuclease, Artemis, as a PTIP-binding protein PMID: 25512557
- the 5'-exonuclease is intrinsic to ARTEMIS, making it relevant to the role of ARTEMIS in nonhomologous DNA end joining PMID: 24500713
- DNA ligase IV and Artemis act cooperatively to promote nonhomologous end-joining PMID: 23967291
- 2 siblings are described with combined immunodeficiency (CID) and immunodysregulation caused by compound heterozygous Artemis mutations. PMID: 24230999
- Artemis levels significantly influence radiation toxicity in human cells PMID: 22713703
- Our findings indicate a novel function of Artemis as a molecular switch that converts stalled replication forks harboring single-stranded gap DNA lesions into double-strand breaks, thereby activating the ATM signaling pathway PMID: 23465063
- Structural basis of DNA ligase IV-Artemis interaction in nonhomologous end-joining. PMID: 23219551
- these results suggest that Artemis functions as a positive regulator of AMPK signaling by stabilizing the LKB1-AMPK complex. PMID: 23044421
- study identified a new SCID mutation in a consanguineous Israeli Arab family; sequencing identified an 8 bp insertion in exon 14 (1306ins8) of DCLRE1C in all affected patients; this causes an alteration in amino acid 330 of the protein from cysteine to a stop codon (p.C330X) PMID: 22527898
- Results show that during S-phase Artemis but not ATM is dispensable for homologous recombination of radiation-induced double-strand breaks. PMID: 22730303
- Regulation of p27 by Artemis and DDB2 is important for cell cycle progression in normally proliferating cells. PMID: 22134138
- Point mutations in Artemis that disrupt its interaction with Ligase IV or DNA-PKcs reduce V(D)J recombination. PMID: 22529269
- The dominant negative mutant Artemis fragment (D37N-413aa) enhanced tumor cell radiosensitivity through blocking activity of endogenous Artemis and DNA repair. PMID: 21641068
- antisense oligonucleotide (AON) covering the intronic mutation restored WT Artemis transcript levels and non-homologous end-joining pathway activity in the patient fibroblasts PMID: 21390052
- analysis of differences in sensitivity to DNA-damaging Agents between XRCC4- and Artemis-deficient human cells PMID: 21785230
- Artemis is required for the repair of DNA double strand breaks that arise endogenously or following oxidative stress. PMID: 21596788
- Restoration of chemo/radioresistance by wild-type, but not D165N Artemis suggests that the lack of endonucleolytic trimming of DNA ends is the principal cause of sensitivity to double-strand cleaving agents in Artemis-deficient cells. PMID: 21531702
- Studies indicate that codon-based models of gene evolution yielded statistical support for the recurrent positive selection of five NHEJ genes during primate evolution: XRCC4, NBS1, Artemis, POLlambda, and CtIP. PMID: 20975951
- Functional analyses on patients' fibroblasts demonstrated that the corresponding alleles carry null mutations of the DCLRE1C gene. PMID: 19953608
- Plays role in the 3'-processing reaction and protection of the ends of viral DNA (HIV-1) after reverse transcription. Involved in multiple steps including integration and pre-integration steps during retroviral replication. PMID: 20003485
- DNA-PKcs regulates Artemis by both phosphorylation and complex formation to permit enzymatic activities that are critical for the hairpin-opening step of V(D)J recombination and for the 5' and 3' overhang processing in nonhomologous DNA end joining. PMID: 11955432
- A nonsense founder mutation discovered in exon 8 of Artemis results in the truncation of the deduced protein product and indicates that the SNM1-like gene (Artemis) is the gene responsible for SCID in Athabascan-speaking Native Americans. PMID: 12055248
- deletions and missense mutations in the Artemis gene can cause radiosensitive-SCID with defective coding joint formation and lead to an early and complete B-cell differentiation block PMID: 12406895
- Artemis has a role in T and B lymphocyte immunodeficiency and in predisposition to lymphoma through the NHEJ pathway of DNA repair PMID: 12569164
- the genomic exon 3 deletion is unique to Japan and may be considered as a founder haplotype. PMID: 12592555
- Properties of the Artemis proteins are integrated into the processes of V(D)J recombination and non-homologous end-joining factors. PMID: 14628082
- Artemis uses one or two Zn(II) ions to exert its catalytic activity, like bacterial class B beta-Lact enzymes hydrolyzing beta-lactam compounds. PMID: 14744996
- The hairpin-opening activity of ARTEMIS and/or its overhang endonucleolytic activity are necessary but its exonuclease activity is not sufficient for the process of V(D)J recombination. PMID: 15071507
- Data show that Artemis interacts with cell cycle checkpoint proteins and is a phosphorylation target of the checkpoint kinases ATM or ATR after exposure of cells to IR or UV irradiation, respectively. PMID: 15456891
- Atemis is an effector of dna repair that can be phosphorylated by ataxia-telangiectasia-mutated kinase (ATM) and possibly by DNA-dependent protein kinase catalytic subunit and ATM-and Rad3-related kinase depending on the type of DNA damage. PMID: 15468306
- ATM, Artemis, and proteins locating to gamma-H2AX foci have roles in double-strand break rejoining PMID: 15574327
- our finding places Artemis at the signaling crossroads downstream of DNA-PKcs and ATM in IR-induced DSB repair. PMID: 15723659
- report on a first patient with clinical and immunologic features of OS caused by hypomorphic ARTEMIS mutations. Sequencing of the ARTEMIS gene revealed a compound heterozygosity in this nonhomologous end-joining (NHEJ) factor PMID: 15731174
- Artemis:DNA-PKcs nuclease may be important in removing secondary structures that hinder processing of DNA ends during nonhomologous DNA end joining . PMID: 15936993
- The uncharacterized C-terminal domain of Artemis has important regulatory roles; results lead to a model for how DNA-PKcs activates Artemis by phosphorylation PMID: 16093244
- characterization of six DNA-PK phosphorylation sites on Artemis whose phosphorylation shows dependence on its association with DNA-PK catalytic subunit and is induced by double-stranded DNA damage PMID: 16600297
- Ku-mediated assembly of DNA-PK on DNA ends is responsible for a dissociation of the DNA-PKcs.Artemis complex. PMID: 16857680
- DNA-PK autophosphorylation regulates Artemis access to DNA ends, providing insight into the mechanism of Artemis mediated DNA end processing. PMID: 16874298
- Artemis efficiently trims long 3'-phosphoglycate-terminated overhangs induced in DNA by radiation and other radical-based toxins. PMID: 17121861
- analyzed the phenotype of cells derived from SCID patients with different mutations in the Artemis gene PMID: 17169382
- ATM regulates G(2)/M checkpoint recovery through inhibitory phosphorylations of Artemis that occur soon after DNA damage, thus setting a molecular switch that, hours later upon completion of DNA repair, allows activation of the Cdk1-cyclin B complex. PMID: 17242184
- There is some sequence-dependent variation in the efficiency and position of hairpin opening by Artemis:DNA-PKcs; providing more clarity on the extent to which the hairpin opening position contributes to junctional diversity in V(D)J recombination. PMID: 17932067
- The Artemis C terminus is essential for V(D)J recombination at the normal Artemis expression level. PMID: 18034425
- H254 plays a key role in the Artemis function, as it is critical for its full activity in vitro. PMID: 19022407
- Results link Artemis to the predominant nonhomologous end-joining pathway during immunoglobulin class switch recombination. PMID: 19075292
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相關疾病：Severe combined immunodeficiency autosomal recessive T-cell-negative/B-cell-negative/NK-cell-positive with sensitivity to ionizing radiation (RSSCID); Severe combined immunodeficiency Athabaskan type (SCIDA); Omenn syndrome (OS)
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亞細胞定位：Nucleus.
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蛋白家族：DNA repair metallo-beta-lactamase (DRMBL) family
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組織特異性：Ubiquitously expressed, with highest levels in the kidney, lung, pancreas and placenta (at the mRNA level). Expression is not increased in thymus or bone marrow, sites of V(D)J recombination.
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