MRE11A编码形成Mre11-Rad50-Nbs（MRN）复合物的蛋白质，参与DNA修复和重组。MRN复合物激活激酶ATM（ataxia telangiectasia mutated）和ATR（ATM-和RAD3-相关）以启动DNA损伤应答并且自身被ATM磷酸化。MRE11A具有对刺激修复很重要的DNA核酸酶活性。MRN复合物的激活使得G2 / M细胞周期检查点能够响应DNA损伤。MRE11A的胚系突变与共济失调-毛细血管扩张样疾病相关，易患乳腺癌和卵巢癌。MRE11A也是家族性乳腺癌和卵巢癌易感基因，并且还在子宫内膜癌，结直肠癌和淋巴癌中检测到突变。MRE11A多态性和突变影响对化学疗法，放射疗法和聚（ADP-核糖）聚合酶（PARP）抑制剂的响应。

RE11A encodes a protein that forms the Mre11-Rad50-Nbs (MRN) complex involved in sensing and repairing DNA double strand breaks. The complex activates the kinases ATM (ataxia telangiectasia mutated) and ATR (ATM- and RAD3-related) to initiate DNA damage responses and is itself phosphorylated by ATM. MRE11A has DNA nuclease activity important for stimulating repair. Activation of the MRN complex enables the G2/M cell cycle checkpoint in response to DNA damage. The complex is also important for genomic integrity at telomeres, replication forks, immunoglobulin gene loci during rearrangements, and DNA breaks formed in meiosis. Activation of repair mechanisms include non-homologous end joining and homologous recombination repair . Mutations in MRE11A are associated with an ataxia-telangiectasia-like disorder, resulting in chromosomal instability and sensitivity to ionizing radiation. MRE11A is also a familial breast and ovarian cancer susceptibility gene and mutations have also been identified in endometrial, colorectal, and lymphoid cancers. MRE11A polymorphisms and mutations affect response to chemotherapy, radiotherapy, and Poly(ADP-ribose) polymerases (PARP) inhibitors.

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