Nijmegen Breakage Syndrome (NBS) is characterized by extreme radiation sensitivity and chromosomal instability. The NBS1 gene product, p95/nibrin/NBS1, forms a complex with Rad50 and Mre11. Cells deficient in this complex have problems with DNA double-strand break repair, cell cycle checkpoint control, and telomere length maintenance. NBS1 contains a forkhead-associated domain (FHA) and a breast cancer carboxy-terminal domain (BRCT) in the N-terminal region. Both of these domains have been found in DNA-damage responsive cell cycle checkpoint proteins. The complex containing NBS1, Rad50, and Mre11 possesses manganese-dependent single stranded DNA endonuclease and 3' to 5' exonuclease activities. In addition, NBS1 is required for DNA-damage dependent phosphorylation of Mre11. This phosphorylation may be required for proper nuclear localization of the NBS1-Rad50-Mre11 complex to sites of DNA double-strand breaks. NBS1 interacts directly with telomere repeat binding factor, TRF1, via its C-terminal region, and both NBS1 and Mre11 co-localize with TRF1 at promyelocytic leukemia nuclear bodies. Thus, the NBS1-Rad50-Mre11 complex may be important for both DNA damage repair, and telomere length maintenance.
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注意事项：
1.Since applications vary, each investigator should titrate the reagent to obtain optimal results.
2.Caution: Sodium azide yields highly toxic hydrazoic acid under acidic conditions. Dilute azide compounds in running water before discarding to avoid accumulation of potentially explosive deposits in plumbing.
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