货号：07-317

品牌：Merck Millipore

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金山科研平台，产品价格货期咨询微信：jinshanbio Description: Anti-Nibrin/Nbs1 View All» Promotional Text: Special Shipping Offer on Antibodies100% Performance Guaranteed View All» Trade Name: Upstate (Millipore) View All» Specificity: Nibrin/Nbs1 View All» Molecular Weight: 95kDa View All» Immunogen: GST fusion protein corresponding to residues 395-742 of human Nibrin/Nbs1/hp95 View All» Isotype: IgG View All» Species Reactivity: Human View All» Quality Assurance: routinely evaluated by immunoblot in Jurkat and A431 cell lysates View All» Purification Method: Antiserum View All» Presentation: antisera with 0.02% sodium azide View All» Storage Conditions: 1 year at 4°C View All» UniProt Number: O60934 View All» Entrez Gene Number: NM_002485 View All» Gene Symbol:

• NBN

• P95

• FLJ10155

• NBS

• AT-V1

• AT-V2

• MGC87362

• ATV

• Nibrin

• NBS1
  View All» Usage Statement: 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. View All» Key Applications:
  • Immunoprecipitation

  • Western Blotting

  • Immunocytochemistry
    View All» Entrez Gene Summary: 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. View All» UniProt Summary: 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. View All» Brand Family: Upstate View All» Product Name: Anti-Nibrin/Nbs1 View All» Antibody Type: Polyclonal Antibody View All» Qty/Pk: 50 µL View All» Format: Serum View All» Host: Rabbit View All»
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