Storage instructionsShipped at 4°C. Store at -20°C.
Storage bufferConstituent: Water
PurityTissue culture supernatant
Our Abpromise guarantee covers the use of ab175800 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|IP||Use at an assay dependent concentration.|
|WB||Use at an assay dependent concentration.|
FunctionComponent 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. Forms a complex with RBBP8 to link DNA double-strand break sensing to resection. Enhances AKT1 phosphorylation possibly by association with the mTORC2 complex.
Tissue specificityUbiquitous. Expressed at high levels in testis.
Involvement in diseaseNijmegen breakage syndrome
Defects in NBN might play a role in the pathogenesis of childhood acute lymphoblastic leukemia (ALL).
Sequence similaritiesContains 1 BRCT domain.
Contains 1 FHA domain.
DomainThe 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.
modificationsPhosphorylated by ATM in response of ionizing radiation, and such phosphorylation is responsible intra-S phase checkpoint control and telomere maintenance.
Cellular localizationNucleus. Nucleus, PML body. Chromosome, telomere. Localizes to discrete nuclear foci after treatment with genotoxic agents.
- Information by UniProt
- AT V1 antibody
- AT V2 antibody
- ATV antibody
Immunocytochemistry/Immunofluorescence analysis of U2OS cells labelling p95 NBS1 with ab175800 at 1:1000. Cells were fixed with PFA and permeabilized with methanol for 2 minutes. Cells were incubated with the primary anitbody overnight at room temperature. Left - p95 NBS1, Middle - DAPI, Right - Merge.
Immunohistochemistry (formalin/PFA-fixed paraffin-embedded) analysis of Human linfoid tissue labelling p95 NBS1 with ab175800 at 1:1000. Antigen retrieval was by microwave boiling for 7 minutes in 10mM TrisHCl, pH9. Left - 4X, Right - 20X.
Immunoprecipitation analysis of U2OS cells labelling p95 NBS1 with ab175800. Protein A beads were crosslinked to the serum.
All lanes : Anti-p95 NBS1 antibody (ab175800)
Lane 1 : Control si
Lane 2 : Nbs1 si
Lane 3 : NIH 3T3 cell lysate at 40 µg