|Application ||WB, IHC, IF|
|Calculated MW||84959 Da|
|Other Names||Nibrin, Cell cycle regulatory protein p95, Nijmegen breakage syndrome protein 1, NBN, NBS, NBS1, P95|
|Target/Specificity||A synthetic peptide corresponding to residues near C-terminus of human Nibrin was used as immunogen|
|Format||50 mM Tris-Glycine (pH 7.4), 0.15 M NaCl, 40% Glycerol, 0.01% sodium azide and 0.05% BSA.|
|Storage||Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles.|
|Precautions||Nibrin/NBS1 Antibody is for research use only and not for use in diagnostic or therapeutic procedures.|
|Synonyms||NBS, NBS1, P95|
|Function||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 MRE11. 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.|
|Cellular Location||Nucleus. Nucleus, PML body. Chromosome, telomere Note=Localizes to discrete nuclear foci after treatment with genotoxic agents.|
|Tissue Location||Ubiquitous. Expressed at high levels in testis|
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Provided below are standard protocols that you may find useful for product applications.
Nibrin (also known as NBS1 or p95) contains 2 modules found in cell cycle check point; a forkhead-associated domain & a BRCA1 C-teminal repeat (1). In mammalian cells, Nibrin forms a complex with Mre11 & Rad50 (MRN). Nuclear localization of the MRN complex is relevant for chromosomal stability, meiotic recombination, DNA repair & telomere maintenance in eukaryotic cells(2-4). Mutation in Nibrin & Mre11 causes respectively two related disorders, Nijmegen breakage syndrome (NBS) & Ataxia-Telangiectasia- like disorder (ATLD).
1. Trujillo, K. M., Yuan, S. S., Lee, E. Y., and Sung, P. (1998) J. Biol. Chem. 273, 21447-21450
2. Dolganov, G. M., Maser, R. S., Novikov, A., Tosto, L., Chong, S., Bressan, D. A., and Petrini, J. H. (1996) Mol. Cell. Biol. 16, 4832-4841
3. Zhu, J., Petersen, S., Tessarollo, L. & Nussenzweig, A. (2001) Curr. Biol. 11, 105-109
4. Varon, R., Vissinga, C., Platzer, M., Cerosaletti, K. M., Chrzanowska, K. H., Saar, K., Beckmann, G., Seemanova, E., Cooper, P. R., Nowak, N. J., et al. (1998) Cell 93, 467-476.
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