ATRX Antibody, HRP conjugated

說明書

中文名稱：

ATRX兔多克隆抗體, HRP偶聯
貨號：

CSB-PA002437LB01HU
規格：

￥880
其他：

產品詳情
產品評價
相關產品
靶點詳情

產品詳情

產品名稱：

Rabbit anti-Homo sapiens (Human) ATRX Polyclonal antibody
Uniprot No.：

P46100
基因名：

ATRX
別名：

Alpha thalassemia/mental retardation syndrome X linked homolog antibody; ATP dependent helicase ATRX antibody; ATP-dependent helicase ATRX antibody; ATR2 antibody; Atrx antibody; ATRX_HUMAN antibody; DNA dependent ATPase and helicase antibody; Helicase 2; X linked antibody; MGC2094 antibody; MRXHF1 antibody; RAD54 antibody; RAD54L antibody; SFM1 antibody; SHS antibody; Transcriptional regulator ATRX antibody; X linked helicase II antibody; X linked nuclear protein antibody; X-linked helicase II antibody; X-linked nuclear protein antibody; XH2 antibody; XNP antibody; Znf HX antibody; Znf-HX antibody
宿主：

Rabbit
反應種屬：

Human
免疫原：

Recombinant Human Transcriptional regulator ATRX protein (2161-2443AA)
免疫原種屬：

Homo sapiens (Human)
標記方式：

HRP
克隆類型：

Polyclonal
抗體亞型：

IgG
純化方式：

>95%, Protein G purified
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
Protocols：

ELISA 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：

Involved in transcriptional regulation and chromatin remodeling. Facilitates DNA replication in multiple cellular environments and is required for efficient replication of a subset of genomic loci. Binds to DNA tandem repeat sequences in both telomeres and euchromatin and in vitro binds DNA quadruplex structures. May help stabilizing G-rich regions into regular chromatin structures by remodeling G4 DNA and incorporating H3.3-containing nucleosomes. Catalytic component of the chromatin remodeling complex ATRX:DAXX which has ATP-dependent DNA translocase activity and catalyzes the replication-independent deposition of histone H3.3 in pericentric DNA repeats outside S-phase and telomeres, and the in vitro remodeling of H3.3-containing nucleosomes. Its heterochromatin targeting is proposed to involve a combinatorial readout of histone H3 modifications (specifically methylation states of H3K9 and H3K4) and association with CBX5. Involved in maintaining telomere structural integrity in embryonic stem cells which probably implies recruitment of CBX5 to telomeres. Reports on the involvement in transcriptional regulation of telomeric repeat-containing RNA (TERRA) are conflicting; according to a report, it is not sufficient to decrease chromatin condensation at telomeres nor to increase expression of telomeric RNA in fibroblasts. May be involved in telomere maintenance via recombination in ALT (alternative lengthening of telomeres) cell lines. Acts as negative regulator of chromatin incorporation of transcriptionally repressive histone MACROH2A1, particularily at telomeres and the alpha-globin cluster in erythroleukemic cells. Participates in the allele-specific gene expression at the imprinted IGF2/H19 gene locus. On the maternal allele, required for the chromatin occupancy of SMC1 and CTCTF within the H19 imprinting control region (ICR) and involved in esatblishment of histone tails modifications in the ICR. May be involved in brain development and facial morphogenesis. Binds to zinc-finger coding genes with atypical chromatin signatures and regulates its H3K9me3 levels. Forms a complex with ZNF274, TRIM28 and SETDB1 to facilitate the deposition and maintenance of H3K9me3 at the 3' exons of zinc-finger genes.
基因功能參考文獻：
1. GNA13 and downstream RhoA signaling, along with potentially other ATRX-responsive genes, implicated in the process of ATRX-dependent glioma cell migration PMID: 29535300
2. The metadynamics data provide new insight into roles of water-bridges and the mechanisms through which K9 hydration stabilizes the H3K9me3:ATRXADD PPI, providing context for the high affinity demonstrated between this protein and peptide. PMID: 29730439
3. ATRX (alpha-thalassemia/mental retardation, X-linked) is a potent biomarker of lower-grade gliomas and is related to recurrence of gliomas. PMID: 29404769
4. Results show that the X-linked nuclear protein (ATRX)-Fas death domain-associated protein (DAXX) complex is involved in gene repression and telomere chromatin structure. PMID: 29084956
5. ATRX, NOTCH1 and NOTCH2 expression varies in angiosarcomas and shows significant correlations with site of origin and poor clinical outcome PMID: 28796347
6. Disrupting the ATRX/DAXX complex and inhibiting telomerase activity in telomerase-positive cancer cells lead to the alternative lengthening of telomeres switch. PMID: 27578458
7. Its expressison is not a surrogate for 1p19q codeletion. PMID: 29550880
8. ATRX or DAXX loss was proved to be an independent predictor for OS of PanNETs in a multivariate Cox regression analysis including well-established risk factors; tumor stage and tumor grade. PMID: 28591701
9. ATRX mutation was identified in 2/5 (40%) cases with mosaic staining while one case showed DAXX mutation. All these cases were characterized by distinctly separate immune-negative and positive/intermixed foci. PMID: 26833422
10. ATRX deficiency was mutually exclusive with LOH. Conversely, ATRX-proficient tumours immunoreactive for R132H-mutant isocitrate dehydrogenase 1 (IDH1) showed a high rate (85%) of LOH. PMID: 26016385
11. Nuclear ATRX loss, either complete or heterogeneous, is encountered in a considerable number of high-grade sarcomas with non-specific genetic alterations. PMID: 26291601
12. data suggest that the presence of ATRX at telomeres may have a central role in suppressing deleterious DNA secondary structures that form at transcribed telomeric repeats, and this may account for the increased DNA damage, stalling of replication and homology-directed repair previously observed upon loss of ATRX function PMID: 28487353
13. Both primary Alternative lengthening of telomeres(ALT) -positive and ATRX/DAXX-negative PanNETs are independently associated with aggressive clinicopathologic behavior and displayed reduced recurrence-free survival. In contrast, ALT activation and loss of ATRX/DAXX are both associated with better overall survival in patients with metastases PMID: 27663587
14. Whole-exome sequencing has identified recurrent mutations in the genes DAXX and ATRX, which correlate with loss of protein expression and alternative lengthening of telomeres (ALT).ALT and DAXX/ATRX loss in PanNETs was associated with shorter disease-free survival (DFS) and disease-specific survival (DSS) and likely plays a significant role in driving metastatic disease PMID: 27407094
15. The results of this study support an important role for ATRX loss and acquisition of ALT in the biology of NF1-associated gliomas, particularly diffuse and high-grade tumors developing in adults. PMID: 27650176
16. We propose that mutations in alpha thalassemia-mental retardation syndrome X-linked (ATRX)/death-domain associated protein (DAXX) prime alternative lengthening of telomeres activation by disrupting telomeric heterochromatin. PMID: 28741530
17. Structural and biochemical characterization of DAXX-ATRX interaction. PMID: 28875283
18. Structural basis for DAXX interaction with ATRX. PMID: 28875424
19. Review of research progress of TERT-mediated and ATRX-mediated telomere maintenance and neuroblastoma, especially high-risk tumors PMID: 28452859
20. This study demonstrated the ATRX loss in glioneuronal tumors with neuropil-like islands. PMID: 27469217
21. 6 cases of diffuse glioma that presented a diagnostic challenge due to conflicting IDH1/IDH2, ATRX, and 1p/19q results. PMID: 28549927
22. The chromatin remodeling enzyme ATRX is a regulator of therapy induced senescence and drives cells into this state via multiple mechanisms. PMID: 28855512
23. The present study explored the structural consequences influenced by two observed mutations V194I and C220R on ADD domain of ATRX protein by applying all atom molecular dynamics (MD) simulation. PMID: 28294389
24. Study demonstrate that the retention of ATRX expression in all oligodendroglial tumor nuclei strongly supports the diagnosis of oligodendroglioma. Conversely, its loss in tumor astrocytic nuclei favors the diagnosis of astrocytoma. PMID: 27796734
25. Given the high frequency of ATRX and DAXX mutations in cancer, these chromatin regulators likely play a key role in pathogenesis [review] PMID: 28062559
26. Data suggest that identification of IDH1-R132H and ATRX loss status in the primary-recurrent gliomas may aid in treatment strategy selection, therapeutic trial design, and clinical prognosis evaluation. PMID: 26918938
27. The results suggest that ATRX is required to limit replication stress during cellular proliferation, whereas upregulation of PARP-1 activity functions as a compensatory mechanism to protect stalled forks, limiting genomic damage, and facilitating late-born neuron production. PMID: 27171262
28. Distinct histological and molecular characteristics of adult diffuse gliomas with and without ATRX immunoreactivity indicate the utility of ATRX immunohistochemistry in diagnostic practice. PMID: 26741321
29. ATRX loss can be reliable for the pathogenesis of neuroendocrine pancreatic neoplasms. PMID: 28371511
30. Mutation in ATRX gene is associated with cancer more frequently in males. PMID: 27869828
31. Nek1 phosphorylates Rad54 and regulates Rad51 removal to orchestrate homologous recombination and replication fork stability. PMID: 27264870
32. Results suggest that cell cycle progression and proliferation of HeLa cells can be tightly controlled by the abundance of RAD51 and RAD54 proteins, which are essential for the rapid response to postreplicative stress and DNA damage stress. PMID: 28190324
33. ATRX binds to the 3' exons of zinc finger genes to maintain their genomic stability through preservation of H3K9me3. PMID: 27029610
34. somatic mutations in the ATRX gene have been observed as recurrent alterations in both osteosarcoma and brain tumors. However, it is unclear if there is any association between osteosarcoma and germline ATRX mutations, specifically in patients with constitutional ATR-X syndrome. PMID: 28371217
35. AMab-6 sensitively detects ATRX in Western blot and immunohistochemical analyses, indicating that AMab-6 could become the standard marker to determine the ATRX mutation status of gliomas in immunohistochemical analyses PMID: 27788029
36. our study describes a novel missense mutation of the ATRX gene helicase domain, carried by three affected males of the two generations of the same family and segregated with intellectual deficiency, dysmorphism and behaviour disorder without alpha-thalassaemia and with non-skewed X-chromosome inactivation PMID: 26997013
37. ATRX mutation is associated with increased mutation rate at the single-nucleotide variant (SNV) level. PMID: 26936505
38. ATRX loss may predict better clinical outcome in astrocytoma patients with p53 overexpression as compared to patients with wild-type ATRX PMID: 26395639
39. Loss of ATRX was highly associated with alternative lengthening of telomeres PMID: 26428317
40. For WHO grade II diffuse glioma, molecular classification using 1p/19qcodel, IDHmut, and ATRX loss more accurately predicts outcome and should be incorporated in the neuropathologic evaluation. PMID: 26210286
41. We provide an overview of the individual components (ATRX, DAXX and/or H3.3) tested in each study and propose a model where the ATRX/DAXX chaperone complex deposits H3.3 to maintain the H3K9me3 modification at heterochromatin throughout the genome. PMID: 26773061
42. Frequent ATRX mutations and aberrant ATRX gene expression in uterine leiomyosarcomas. PMID: 26891131
43. Daxx and Atrx safeguard the genome by silencing repetitive elements when DNA methylation levels are low. PMID: 26340527
44. Loss of ATRX is associated with DNA methylation pattern of chromosome end and impacts biological behaviors of astrocytic tumors. PMID: 25971279
45. Data identify MDM2 and ATRX as new regulators controlling geroconversion, the process by which quiescent cells become senescent. PMID: 25803170
46. ATRX interacts with ZNF274, TRIM28 and SETDB1 and binds to the 3' exons of zinc finger genes that present an atypical H3K9me3/H3K36me3 chromatin signature. Depletion of ATRX or ZNF274 leads to decreased H3K9me3 levels at zinc finger genes and other atypical chromatin regions. PMID: 27029610
47. ATRX loss was the most likely mechanism of alternative telomere lengthening in liposarcoma and alternative telomere lengthening was a prognostic factor of poor outcome in dedifferentiated liposarcoma. PMID: 26022452
48. loss of ATRX function cooperates with one or more as-yet unidentified genetic or epigenetic alterations to activate Alternative Lengthening of Telomeres PMID: 26001292
49. This study showed that expression of ectopic ATRX triggers a suppression of the pathway and telomere shortening. PMID: 26143912
50. Co-inheritance of novel ATRX gene mutation and alpha-globin/beta-globin gene mutations has been found in transfusion dependent beta-thalassemia Saudi Arabia patients. PMID: 25976463
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相關疾病：

Alpha-thalassemia mental retardation syndrome, X-linked (ATRX); Mental retardation, X-linked, syndromic, with hypotonic facies 1 (MRXSHF1); Alpha-thalassemia myelodysplasia syndrome (ATMDS)
亞細胞定位：

Nucleus. Chromosome, telomere. Nucleus, PML body.
蛋白家族：

SNF2/RAD54 helicase family
組織特異性：

Ubiquitous.
數據庫鏈接：

HGNC: 886

OMIM: 300032

KEGG: hsa:546

STRING: 9606.ENSP00000362441

UniGene: Hs.533526

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