BCOR Antibody

說明書

中文名稱：

BCOR兔多克隆抗體
貨號：

CSB-PA259179
規格：

￥1100
圖片：
The image on the left is immunohistochemistry of paraffin-embedded Human thyroid cancer tissue using CSB-PA259179(BCOR Antibody) at dilution 1/15, on the right is treated with fusion protein. (Original magnification: ×200)
其他：

產品詳情
產品評價
相關產品
靶點詳情

產品詳情

Uniprot No.：

Q6W2J9
基因名：

BCOR
別名：

5830466J11Rik antibody; 8430401K06Rik antibody; ANOP 2 antibody; ANOP2 antibody; BCL 6 co repressor antibody; BCL 6 corepressor antibody; BCL 6 interacting corepressor antibody; BCL-6 corepressor antibody; BCL6 co repressor antibody; BCL6 corepressor antibody; BCL6 interacting corepressor antibody; BCoR antibody; BCOR_HUMAN antibody; FLJ20285 antibody; FLJ38041 antibody; KIAA1575 antibody; MAA 2 antibody; MAA2 antibody; MCOPS 2 antibody; MCOPS2 antibody; MGC131961 antibody; MGC71031 antibody
宿主：

Rabbit
反應種屬：

Human,Mouse
免疫原：

Fusion protein of Human BCOR
免疫原種屬：

Homo sapiens (Human)
標記方式：

Non-conjugated
抗體亞型：

IgG
純化方式：

Antigen affinity purification
濃度：

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

-20°C, pH7.4 PBS, 0.05% NaN3, 40% Glycerol
產品提供形式：

Liquid
應用范圍：

ELISA,IHC

推薦稀釋比：

Application	Recommended Dilution
ELISA	1:1000-1:5000
IHC	1:10-1:50

Protocols：

ELISA Protocol
Immunohistochemistry (IHC) 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：

Transcriptional corepressor. May specifically inhibit gene expression when recruited to promoter regions by sequence-specific DNA-binding proteins such as BCL6 and MLLT3. This repression may be mediated at least in part by histone deacetylase activities which can associate with this corepressor. Involved in the repression of TFAP2A; impairs binding of BCL6 and KDM2B to TFAP2A promoter regions. Via repression of TFAP2A acts as a negative regulator of osteo-dentiogenic capacity in adult stem cells; the function implies inhibition of methylation on histone H3 'Lys-4' (H3K4me3) and 'Lys-36' (H3K36me2).
基因功能參考文獻：
1. BCOR-ITD was positively detected in the cfDNA of two cases, both of which were later diagnosed as CCSK based on histological feature of the resected tumor specimen, while it was not detected for a normal control and a patient diagnosed with Wilms' tumor. PMID: 30126017
2. Multivariate analysis demonstrated that BCOR mutations were an independent unfavorable prognostic factor (P = 0.0038, P = 0.0463) for both OS and RFS. PMID: 29663558
3. BCOR mutation is associated with tooth agenesis. PMID: 30046887
4. The majority of the cases of clear cell sarcoma of the kidney in our cohort had BCOR internal tandem duplications PMID: 28833375
5. BCOR was negative in all ewing sarcoma family of tumors PMID: 28864350
6. BCOR immunohistochemical staining is a highly sensitive marker for YWHAE-NUTM2 high-grade endometrial stromal sarcoma with both classic and unusual morphology and identifies a subset of high-grade endometrial stromal sarcoma with BCOR alterations, including BCOR rearrangement and internal tandem duplication. PMID: 28621321
7. the frequent BCOR mutations and the absence of alterations in genes regulating the NF-kappaB pathway (triple-negative for KLF2, TNFAIP3 and MYD88 mutations) or the absence of a BRAF mutation appear to delineate a specific genetic pattern of SDRPL, which is distinct from that already identified in SMZL, HCL or HCL-v. PMID: 28751561
8. BCOR internal tandem duplication and/or nuclear immunoreactivity for BCOR or BCL6 can aid in the diagnosis of primitive myxoid mesenchymal tumor of infancy and help to differentiate it from congenital infantile fibrosarcoma. PMID: 28256570
9. Case Report: altered BCOR expression and homogeneous coactivation of both the SHH and WNT signaling pathways, and suggest potential novel therapeutic approaches for patients with a high grade neuroepithelial tumor of the central nervous system with BCOR alteration diagnosis. PMID: 27825128
10. Immunohistochemistry for either CCNB3 or BCOR is not completely sensitive and specific in undifferentiated sarcoma with BCOR-CCNB3 fusion. PMID: 28877060
11. Case Reports: renal sarcomas with BCOR-CCNB3 gene fusion showing histological overlap with BCOR-related clear cell sarcoma of the kidney. PMID: 28817404
12. we report recurrent BCOR exon 16 internal tandem duplications and YWHAE-NUTM2B fusions in half of infantile soft tissue undifferentiated round cell sarcoma and most primitive myxoid mesenchymal tumor of infancy cases, but not in other pediatric sarcomas. PMID: 26945340
13. All small blue round cell tumors (SBRCTs) with BCOR-MAML3 and BCOR-CCNB3 fusions, as well as most with BCOR internal tandem duplications (93%), and all clear cell sarcoma of kidney showed strong and diffuse nuclear BCOR immunoreactivity. PMID: 27428733
14. we find that ESS with ZC3H7B-BCOR fusion constitutes a novel type of high-grade endometrial stromal sarcoma and shares significant morphologic overlap with myxoid leiomyosarcoma PMID: 27631520
15. We report the case of a 7-month-old girl with atypical oculo-facio-cardio-dental syndrome (OFCD). A novel de novo pathogenic mutation in the BCL6 interacting co-repressor gene (BCOR) (c.4540C>T; p.Arg1514*), was identified on the X chromosome PMID: 28317252
16. ZC3H7B-BCOR and MEAF6-PHF1 fusions occurred predominantly in S100 protein-negative and malignant ossifying fibromyxoid tumors. PMID: 24285434
17. Given the high frequency and pattern of aberration, BCOR is likely to play an important role in ENKTL pathogenesis as a tumor suppressor gene PMID: 26773734
18. Data suggest the detection of the partial duplication inside exon 15 of proto-oncogene protein BCOR as part of the diagnostic process of pediatric kidney tumors. PMID: 26516930
19. Report mutually exclusive BCOR internal tandem duplications and YWHAE-NUTM2 fusions in clear cell sarcoma of kidney. PMID: 27000436
20. The present study further expands the genetic spectrum of small blue round cell tumors, with 2 novel fusions, BCOR-MAML3 and ZC3H7B-BCOR, identified PMID: 26752546
21. Somatic internal tandem duplications (ITDs) clustering in the C terminus of BCOR were found in 85% of pediatric clear cell sarcoma of the kidney. Mutant BCOR transcripts and proteins are markedly upregulated in ITD-positive tumours. PMID: 26573325
22. This study adds to recent reports on the clinicopathologic spectrum of BCOR-CCNB3 fusion-positive sarcomas, a newly emerging entity within the undifferentiated unclassified sarcoma category PMID: 25360585
23. Using reported human BCOR mutations in patients with oculofaciocardiodental syndrome, the authors identified nuclear localization signals at two possible sites; at aa1131-1141 (NLS1) and at aa1158-1167(NLS2). PMID: 26054978
24. study concluded that in pediatric acute myeloid leukemia, BCOR and BCORL1 mutations rarely occur PMID: 25596268
25. Data indicate that the internal tandem duplications in the BCOR gene (BCL6 corepressor) affecting the C terminus in 100% (20/20) of clear cell sarcoma of the kidney (CCSK) tumors PMID: 26098867
26. The nonsense and frameshift mutations, which introduce premature termination codons, were found to contribute to oculofaciocardiodental syndrome in our two patients. PMID: 24694763
27. report of the clinical and molecular data of 3 new patients with oculofaciocardiodenta and review the literature for all published patients and their mutations in the BCOR gene PMID: 23557072
28. findings identify the BCL6/BCOR/SIRT1 complex as a potent repressor of the SHH pathway in normal and oncogenic neural development PMID: 25490446
29. Study provides a detailed description of the histologic spectrum, immunohistochemical features, and clinical characteristic of BCOR-CCNB3 sarcoma justifying distinction from Ewing sarcoma with its typical EWS/FUS-ETS translocations. PMID: 24805859
30. two components of a transcriptional repressor complex (BCL-6 and BCoR) of wildtype amino acid sequence can independently or jointly induce the formation of nuclear aggregates when overexpressed. PMID: 24146931
31. ZC3H7B-BCOR and MEAF6-PHF1 fusions occurred predominantly in S100 protein-negative and malignant OFMT. PMID: 24285434
32. Data indicate that sequencing of BCOR and related BCORL1 genes in a cohort of 354 myelodysplastic syndromes (MDS) patients identified 4.2% and 0.8% of mutations respectively. PMID: 24047651
33. BCL6 corepressor, BCOR, binds the RING finger- and WD40-associated ubiquitin-like (RAWUL) domain of PCGF1 (NSPC1) and PCGF3 but not of PCGF2 (MEL18) or PCGF4 (BMI1). PMID: 23523425
34. FBXL11 inhibited osteo/dentinogenic differentiation potential in MSC cells by associating with BCOR, then increasing histone K4/36 methylation in Epiregulin promoter to repress Epiregulin transcription. PMID: 23074094
35. the mutation in BCOR is likely to be the major determinant for the phenotypes in this Oculofaciocardiodental syndrome (OFCD) family. PMID: 22301464
36. A new fusion was observed between BCOR (encoding the BCL6 co-repressor) and CCNB3 (encoding the testis-specific cyclin B3) on the X chromosome. PMID: 22387997
37. BCOR is a fusion partner of retinoic acid receptor alpha in a t(X;17)(p11;q12) variant of acute promyelocytic leukemia. BCOR-RARA possesses common features with other RARA fusion proteins. PMID: 20807888
38. Our results for the first time implicate BCOR in normal karyotype acute myeloid leukemia pathogenesis. PMID: 22012066
39. The forkhead transcription factor AFX activates apoptosis by induction of the BCL-6 transcriptional repressor. PMID: 11777915
40. role as a key transcriptional regulator during early embryogenesis PMID: 15004558
41. data confirm that BCOR is the causative gene for oculo-facio-cardio-dental syndrome, two small deletions (c.2488_2489delAG and c.3286delG) and a submicroscopic deletion of about 60 kb encompassing at least BCOR exons 2-15. PMID: 15770227
42. BCOR complex components and mono-ubiquitylated H2A localize to BCL6 targets, indicating that the BCOR complex employs Polycomb Group proteins to expand the repertoire of enzymatic activities that can be recruited by BCL6. PMID: 16943429
43. BCOR left-sided embryonic expression is required for vertebrate laterality determination PMID: 17517692
44. The structure of a BCOR corepressor peptide in complex with the BCL6 BTB domain dimer is reported. PMID: 18280243
45. Data show that AP-2alpha was identified as a repressive target of BCOR, and BCOR mutation resulted in abnormal activation of AP-2alpha. PMID: 19578371
46. Function:BCoR is a transcriptional corepressor for BCL-6. Nomenclature:BCoR (BCL-6 corepressor) PMID: 10898795
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相關疾病：

Microphthalmia, syndromic, 2 (MCOPS2)
亞細胞定位：

Nucleus.
蛋白家族：

BCOR family
組織特異性：

Ubiquitously expressed.
數據庫鏈接：

HGNC: 20893

OMIM: 300166

KEGG: hsa:54880

STRING: 9606.ENSP00000367705

UniGene: Hs.659681

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