MRN complex
The MRN complex (MRX complex in yeast) is a protein complex consisting of Mre11, Rad50 and Nbs1 (also known as Nibrin [1] in humans and as Xrs2 in yeast). In eukaryotes, the MRN/X complex plays an important role in the initial processing of double-strand DNA breaks prior to repair by homologous recombination or non-homologous end joining. The MRN complex binds avidly to double-strand breaks both in vitro and in vivo and may serve to tether broken ends prior to repair by non-homologous end joining or to initiate resection prior to repair by homologous recombination. The MRN complex also participates in activating the checkpoint kinase ATM in response to DNA damage.[2][3] Production of short single-strand oligonucleotides by Mre11 endonuclease activity has been implicated ATM activation by the MRN complex.[4]
Role in human disease
Mutations in the human Nbs1 subunit of the MRN complex have been implicated in the rare genetic disorder Nijmegen Breakage Syndrome.[5]
References
- ^ "Atlas of Genetics and Cytogenetics in Oncology and Haematology - NBS1". Retrieved 2008-02-12.
- ^ Lee, JH; Paull, TT (2004 Apr 2). "Direct activation of the ATM protein kinase by the Mre11/Rad50/Nbs1 complex.". Science (New York, N.Y.) 304 (5667): 93–6. PMID 15064416.
- ^ Lee, JH; Paull, TT (2005 Apr 22). "ATM activation by DNA double-strand breaks through the Mre11-Rad50-Nbs1 complex.". Science (New York, N.Y.) 308 (5721): 551–4. PMID 15790808.
- ^ Jazayeri A, Balestrini A, Garner E, Haber JE, Costanzo V (2008). "Mre11-Rad50-Nbs1-dependent processing of DNA breaks generates oligonucleotides that stimulate ATM activity". The EMBO Journal 27 (14): 1953–1962. doi:10.1038/emboj.2008.128. PMID 18596698.
- ^ "eMedicine - Nijmegen Breakage Syndrome". Retrieved 2008-02-12.
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