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merck millipore,默克密理博,05-616,Anti-Nibrin/Nbs1 Antibody, clone EE15
产品名称：Anti-Nibrin/Nbs1 Antibody, clone EE15
产品型号：05-616
Anti-Nibrin/Nbs1 Antibody, clone EE15 is a high quality Mouse Monoclonal Antibody for the detection of Nibrin/Nbs1 & has been validated in IP & WB.
merck millipore,默克密理博,05-616,Anti-Nibrin/Nbs1 Antibody, clone EE15
- 产品介绍
重要规格表
品种反应性 主要应用 宿主 格式 抗体类型 H WB M Purified Monoclonal Antibody 描述 产品目录编号 05-616 Replaces 04-236 品牌系列 Upstate
商名 - Upstate
描述 Anti-Nibrin/Nbs1 Antibody, clone EE15 产品信息 格式 Purified 演示 100mM Tris-glycine, pH 7.4, 0.15M NaCl, 0.05% sodium azide before the addition of glycerol to 30% 应用 应用 Anti-Nibrin/Nbs1 Antibody, clone EE15 is a high quality Mouse Monoclonal Antibody for the detection of Nibrin/Nbs1 & has been validated in IP & WB. 主要应用 - Western Blotting
生物信息 免疫原品种 GST fusion protein corresponding to residues 420-774 of human Nibrin/Nbs1 克隆 clone EE15 宿主 Mouse 特异性 Nibrin/Nbs1 同种型 IgG 品种反应性 Human 抗体类型 Monoclonal Antibody Entrez基因编号 - NM_002485
Entrez基因汇总 Mutations in this gene are associated with Nijmegen breakage syndrome, an autosomal recessive chromosomal instability syndrome characterized by microcephaly, growth retardation, immunodeficiency, and cancer predisposition. The encoded protein is a member of the MRE11/RAD50 double-strand break repair complex which consists of 5 proteins. This gene product is thought to be involved in DNA double-strand break repair and DNA damage-induced checkpoint activation. Two alternatively spliced transcript variants encoding different isoforms have been found for this gene. 基因符号 - NBS1
- NBN
- P95
- MGC87362
- Nibrin
- FLJ10155
- ATV
- AT-V2
- NBS
- AT-V1
纯化方法 Protein G chromatography UniProt编号 - O60934
UniProt汇总 FUNCTION: SwissProt: O60934 # Component of the MRE11/RAD50/NBN (MRN complex) which plays a critical role in the cellular response to DNA damage and the maintenance of chromosome integrity. The complex is involved in double-strand break (DSB) repair, DNA recombination, maintenance of telomere integrity, cell cycle checkpoint control and meiosis. The complex possesses single-strand endonuclease activity and double-strand-specific 3'-5' exonuclease activity, which are provided by MRE11A. RAD50 may be required to bind DNA ends and hold them in close proximity. NBN modulate the DNA damage signal sensing by recruiting PI3/PI4-kinase family members ATM, ATR, and probably DNA-PKcs to the DNA damage sites and activating their functions. It can also recruit MRE11 and RAD50 to the proximity of DSBs by an interaction with the histone H2AX. NBN also functions in telomere length maintenance by generating the 3' overhang which serves as a primer for telomerase dependent telomere elongation. NBN is a major player in the control of intra-S-phase checkpoint and there is some evidence that NBN is involved in G1 and G2 checkpoints. The roles of NBS1/MRN encompass DNA damage sensor, signal transducer, and effector, which enable cells to maintain DNA integrity and genomic stability.
SIZE: 754 amino acids; 84959 Da
SUBUNIT: Component of the MRN complex composed of two heterodimers RAD50/MRE11A associated with a single NBN. Component of the BASC complex, at least composed of BRCA1, MSH2, MSH6, MLH1, ATM, BLM, RAD50 and MRE11A (By similarity). Interacts with histone H2AFX this requires phosphorylation of H2AFX on 'Ser-139'. Interacts with TERF2 and with KPNA2.
SUBCELLULAR LOCATION: Nucleus (By similarity). Note=Localizes to discrete nuclear foci after treatment with genotoxic agents (By similarity).
TISSUE SPECIFICITY: Ubiquitous. Expressed at high levels in testis.
DOMAIN: SwissProt: O60934 The FHA and BRCT domains are likely to have a crucial role for both binding to histone H2AFX and for relocalization of MRE11/RAD50 complex to the vicinity of DNA damage. & The C-terminal domain contains a MRE11-binding site, and this interaction is required for the nuclear localization of the MRN complex. & The EEXXXDDL motif at the C-terminus is required for the interaction with ATM and its recruitment to sites of DNA damage and promote the phosphorylation of ATM substrates, leading to the events of DNA damage response.
PTM: Phosphorylated by ATM in response of ionizing radiation, and such phosphorylation is responsible intra-S phase checkpoint control and telomere maintenance.
DISEASE: SwissProt: O60934 # Defects in NBN are the cause of Nijmegen breakage syndrome (NBS) [MIM:251260]. NBS is an autosomal recessive syndrome characterized by chromosomal instability, radiation sensitivity, microcephaly, growth retardation, immunodeficiency and predisposition to cancer, particularly to lymphoid malignancies. & Defects in NBN are a cause of genetic susceptibility to breast cancer (BC) [MIM:114480]. BC is an extremely common malignancy, affecting one in eight women during their lifetime. A positive family history has been identified as major contributor to risk of development of the disease, and this link is striking for early-onset breast cancer. & Defects in NBN may be associated with aplastic anemia [MIM:609135]. Aplastic anemia is a disease of bone-marrow failure characterized by peripheral pancytopenia and marrow hypoplasia. Most of the cases of aplastic anemia are idiopathic, some are familial and some are due to a viral infection or to exposure to chemicals and radiation. & Defects in NBN might play a role in the pathogenesis of childhood acute lymphoblastic leukemia (ALL).
SIMILARITY: Contains 1 BRCT domain. & Contains 1 FHA domain.
MISCELLANEOUS: In case of infection by adenovirus E4, the MRN complex is inactivated and degraded by viral oncoproteins, thereby preventing concatenation of viral genomes in infected cells.产品使用声明 质量保证 routinely evaluated by immunoblot on Jurkat, HeLa nuclear extract, HeLa whole cell, and A431+/- EGF lysates 使用声明 - Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
储存和货运信息 存储条件 2 years at -20°C 包装信息 数量 200 µg
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