NHEJ1 Recombinant Monoclonal Antibody

說明書

中文名稱：

NHEJ1 Recombinant Monoclonal Antibody
貨號：

CSB-RA187074A0HU
規格：

￥1320
圖片：
Western Blot
Positive WB detected in: HT-29 whole cell lysate(30μg), PC-3 whole cell lysate(30μg), Hela whole cell lysate(30μg), Jurkat whole cell lysate(30μg), A431 whole cell lysate(30μg), SH-SY5Y whole cell lysate(30μg), HepG2 whole cell lysate(30μg)
All lanes:NHEJ1 antibody at 1:1000
Secondary
Goat polyclonal to rabbit IgG at 1/40000 dilution
Predicted band size: 33 kDa
Observed band size: 39 kDa
Exposure time：20s

IHC image of CSB-RA187074A0HU diluted at 1:100 and staining in paraffin-embedded human small intestine tissue performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a Goat anti-rabbit polymer IgG labeled by HRP and visualized using 0.05% DAB.

Overlay Peak curve showing SH-SY5Y cells stained with CSB-RA187074A0HU (red line) at 1:100. The cells were fixed in 4% formaldehyde and permeated by 0.2% TritonX-100 for?10min. Then 10% normal goat serum to block non-specific protein-protein interactions followed by the antibody (1ug/1*10⁶cells) for 45min at 4℃. The secondary antibody used was FITC-conjugated goat anti-rabbit IgG (H+L) at 1/200 dilution for 35min at 4℃.Control antibody (green line) was Rabbit IgG (1ug/1*10⁶cells) used under the same conditions. Acquisition of >10,000 events was performed.
其他：

產品詳情
產品評價
相關產品
靶點詳情

產品詳情

Uniprot No.：

Q9H9Q4
基因名：

NHEJ1
別名：

Non-homologous end-joining factor 1 (Protein cernunnos) (XRCC4-like factor), NHEJ1, XLF
反應種屬：

Human
免疫原：

A synthesized peptide from human NHEJ1 protein
免疫原種屬：

Homo sapiens (Human)
標記方式：

Non-conjugated
克隆類型：

Monoclonal
抗體亞型：

Rabbit IgG
純化方式：

Affinity-chromatography
克隆號：

12H12
濃度：

It differs from different batches. Please contact us to confirm it.
保存緩沖液：

Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, PH 7.4
產品提供形式：

Liquid
應用范圍：

ELISA, WB, IHC, FC

推薦稀釋比：

Application	Recommended Dilution
WB	1:500-1:2000
IHC	1:50-1:200
FC	1:50-1:200

Protocols：

ELISA Protocol
Western Blotting (WB) Protocol
Immunohistochemistry (IHC) Protocol
Flow cytometry (FC) Protocol
儲存條件：

Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
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.
用途：

For Research Use Only. Not for use in diagnostic or therapeutic procedures.

產品評價

靶點詳情

功能：

DNA repair protein involved in DNA non-homologous end joining (NHEJ); required for double-strand break (DSB) repair and V(D)J recombination. Plays a key role in NHEJ by promoting the ligation of various mismatched and non-cohesive ends. Together with PAXX, collaborates with DNA polymerase lambda (POLL) to promote joining of non-cohesive DNA ends. May act in concert with XRCC5-XRCC6 (Ku) to stimulate XRCC4-mediated joining of blunt ends and several types of mismatched ends that are non-complementary or partially complementary. Associates with XRCC4 to form alternating helical filaments that bridge DNA and act like a bandage, holding together the broken DNA until it is repaired. The XRCC4-NHEJ1/XLF subcomplex binds to the DNA fragments of a DSB in a highly diffusive manner and robustly bridges two independent DNA molecules, holding the broken DNA fragments in close proximity to one other. The mobility of the bridges ensures that the ends remain accessible for further processing by other repair factors. Binds DNA in a length-dependent manner.
基因功能參考文獻：
1. this study shows loss of NHEJ1 protein due to a novel splice site mutation in a family presenting with combined immunodeficiency, microcephaly, and growth retardation PMID: 28741180
2. Phospho-blocking and -mimicking mutations impact both the stability and DNA bridging capacity of XRCC4/XLF complexes, but without affecting their ability to stimulate LIG4 activity. Implicit in this finding is that phosphorylation may regulate DNA bridging by XRCC4/XLF filaments. PMID: 28500754
3. Chemotherapy-induced overexpression of XLF and XLF-mediated enhancements in NHEJ activity contribute to chemoresistance in hepatocellular carcinoma (HCC) cells and patients with HCC. Targeting XLF to modulate DSB repair could enhance drug sensitivity and may be a therapeutically useful addition to conventional therapy PMID: 28526069
4. Although PAXX-deficient cells lack c-NHEJ phenotypes, PAXX forms a stable ternary complex with Ku bound to DNA. Thus, PAXX plays an accessory role during c-NHEJ that is largely overlapped by XLF's function. PMID: 27705800
5. The role of XLF in NHEJ is summarized. PMID: 28846869
6. XLF has an important role during V(D)J recombination. PMID: 27281794
7. using dual- and quadruple-trap optical tweezers combined with fluorescence microscopy, we show how human XRCC4, XLF and XRCC4-XLF complexes interact with DNA in real time PMID: 27437582
8. The data suggest that XLF has multiple functions in DNA repair, and they offer potential explanations for the pleiotropic phenotypes associated with XLF deficiency. PMID: 26100018
9. PC4 protects esophageal squamous cell carcinoma cells from IR-induced death by enhancing the nonhomologous end joining-promoting activity of XLF. PMID: 25321468
10. Phosphorylation of XLF impairs non-homologous end-joining DNA repair. PMID: 25661488
11. Werner syndrome protein positively regulates XRCC4-like factor transcription. PMID: 24626809
12. Human XLF is a non-essential, but critical, classic non-homologous end-joining -repair factor. PMID: 24461734
13. An induced pluripotent stem cell (iPSC) model of XLF deficiency, which accurately replicates the double-strand break repair deficiency observed in XLF syndrome patients, is reported. PMID: 23818183
14. Data indicate that Ku70/Ku80 facilitates the cooperative binding of multiple XRCC4/Ligase IV (XL) and XLF molecules to DNA. PMID: 23620595
15. XRCC4 and XLF form long helical protein filaments suitable for DNA end protection and alignment to facilitate DNA double strand break repair. (Review) PMID: 23442139
16. Cernunnos deficiency results in chronic activation of the DNA damage response, P53-driven upregulation of proapoptotic factors, leading to decreased thymocyte viability and a qualitative alteration of the T cell repertoire in both humans and mice. PMID: 23207905
17. Regulation of Double Strand Break repair by EGFR involves both the NHEJ and HR pathway, and appears to occur in most tumor cell lines regardless of p53 and K-Ras mutation status. PMID: 21665306
18. Evidence for how XRCC4-XLF complexes robustly bridge DNA molecules. PMID: 22287571
19. A suggested link between defects in the Cernunnos-dependent nonhomologous end-joining pathway and aberrant class switch recombination or switch translocations during the development of B cell malignancies. PMID: 22312109
20. Multiple truncations of the XLF and XRCC4 proteins were cocrystallized, but yielded low-resolution diffraction (~20 A) PMID: 22102241
21. Data show that XLF and XRCC4 dimers interact through their head domains and form an alternating left-handed helical structure with polypeptide coiled coils and pseudo-dyads of individual XLF and XRCC4 dimers at right angles to the helical axis. PMID: 21936820
22. molecular mechanism for XLF-XRCC4 stimulation of DNA ligation. PMID: 21775435
23. X-ray structure reveals a filament arrangement for XRCC4(1-157) and Cernunnos(1-224) homodimers mediated by repeated interactions through their N-terminal head domains. PMID: 21768349
24. Data show that the heterodimeric domain of Ku was sufficient for the recruitment of XLF to DSBs and for the interaction of Ku with XLF. PMID: 21349273
25. NHEJ in human embryonic stem cells is largely independent of ATM, DNA-PKcs, and PARP but dependent on XRCC4 with repair fidelity several-fold greater than in astrocytes. PMID: 20844317
26. Patients with NBS-like phenotypes may have mutations in the NHEJ1 gene including multiexon deletions, and show that considerable clinical variability could be observed even within the same family. PMID: 20597108
27. identified three amino acids (Arg(64), Leu(65), and Leu(115)) essential for the interaction with X4 and the proper function of Cernunnos PMID: 20558749
28. a natural mutator variant of human DNA polymerase lambda promotes chromosomal instability by compromising NHEJ PMID: 19806195
29. Cernunnos-XLF is a new Non-homologous End Joining pathway protein, which if mutated results in several conditions -immunodeficiency and developmental anomalies and other conditions, which likely result from inability to repair spontaneous DNA damage. PMID: 16439204
30. Studies on the binding of XLF and its role in DNA repair. PMID: 16439205
31. Cernunnos physically interacts with the XRCC4 x DNA-ligase IV complex PMID: 16571728
32. DNA repair protein involved in lymphocyte activation and cell division. PMID: 16828027
33. Results show that a truncated transcript of NHEJ1 is expressed in the polymicrogyric patient cells, suggesting a potential dominant negative effect possibly leading to a different phenotype. PMID: 17191205
34. mutant protein retained its ability to stimulate XRCC4.DNA ligase IV but failed to translocate to the nucleus, and this appears to be the basis for the non-homologous DNA end joining defect in this patient PMID: 17317666
35. Cernunnos/XRCC4-like factor promotes a mismatched end (MEnd) DNA ligase activity to facilitate joining and to preserve DNA sequence. PMID: 17470781
36. Review focuses on the proteins involved in the NHEJ pathway, the major pathway for repair of DNA double-strand breaks, which can become molecular targets in the treatment of cancer. PMID: 17504121
37. Data shows that an intact XRCC4/ligase IV complex is necessary for Cernunnos-XLF mobilization to damaged chromatin. PMID: 17720816
38. The XLF dimer adopts a similar overall structure to that of the XRCC4 dimer, supporting the contention that these two factors are related in function and have arisen from a common evolutionary ancestor. PMID: 18046455
39. Mutational analysis of XLF and XRCC4 reveals a potential interaction interface, suggesting a mechanism for how XLF stimulates the ligation of mismatched ends. PMID: 18158905
40. REVIEW: Role and regulation of XLF PMID: 18335491
41. The major phosphorylation sites in XLF, serine 245 is phosphorylated by DNA-PK, while serine 251 is phosphorylated by Ataxia-Telangiectasia Mutated (ATM), in vivo. PMID: 18644470
42. Results implicate XLF as a C-NHEJ factor but also indicate that developing mouse lymphocytes harbor cell-type-specific factors/pathways that compensate for the absence of XLF function during V(D)J recombination. PMID: 18775323
43. the heads and coiled-coil regions of Cernunnos and X4 are not interchangeable, and they suggest specific roles for each in NHEJ PMID: 19103754
44. role of Cernunnos/XLF in repair of DSBs and maintenance of genomic stability under replication stress conditions PMID: 19223975
45. Recombinant Cernunnos protein restored gap filling and end joining of partially complementary overhangs, and stimulated joining of cohesive ends more than twentyfold. PMID: 19420065
46. The XLF-encoded protein (XRRC4 like factor, FLJ12610) is involved in DNA double-strand break repair via nonhomologous end-joining and associates with the Ligase IV-XRCC4 complex. XLF is mutated in a number of radiosensitive and immuno-deficient patients. PMID: 16439205
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相關疾病：

Severe combined immunodeficiency due to NHEJ1 deficiency (NHEJ1-SCID)
亞細胞定位：

Nucleus. Chromosome.
蛋白家族：

XLF family
組織特異性：

Ubiquitously expressed.
數據庫鏈接：

HGNC: 25737

OMIM: 611290

KEGG: hsa:79840

STRING: 9606.ENSP00000349313

UniGene: Hs.225988

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