中文摘要：

NBS1基因突变可导致一种以染色质不稳定为特征的Nijmegen断裂综合征（NBS）。研究发现NBS1蛋白可与Mre11和Rad50蛋白形成一个Mre11-Rad50-NBS1复合物,在DNA的复制和DNA双链断裂的修复等过程中均起重要作用。本文对NBS1的结构及其在DNA的复制、维持染色质稳定以及DNA损伤应激反应中的作用进行综述。

英文摘要：

NBSI gene mutations can induce a Nijmegen breakage syndrome （NBS） characterized with genome instability. It has been shown that NBS1 protein can bind to Mrel 1 and Rad50 proteins to form a Mrel 1-RadS0-NBS1 （MRN） complex, which plays a vital role in DNA replication and DNA double strand breaks （DSB） repair. This review briefly summarizes the current progress in the functions of NBS1 in DNA replication, DSB repair, cell cycle control, as well as maintenance of genome stability.