Research Progress in the Roles of MRE11-RAD50-NBS1 Complex and Human Diseases
10.3881/j.issn.1000-503X.15556
- VernacularTitle:MRE11-RAD50-NBS1复合物的功能与人类疾病的研究进展
- Author:
Xiaohui XU
1
;
Yidan LIU
Author Information
1. 浙江大学医学院附属妇产科医院, 杭州 310006
- Keywords:
DNA repair;
DNA double-strand break;
homologous recombination;
MRE11-RAD50-NBS1 complex
- From:
Acta Academiae Medicinae Sinicae
2024;46(2):232-241
- CountryChina
- Language:Chinese
-
Abstract:
DNA is susceptible to various factors in vitro and in vivo and experience different forms of damage,among which double-strand break(DSB)is a deleterious form.To maintain the stability of genetic in-formation,organisms have developed multiple mechanisms to repair DNA damage.Among these mechanisms,ho-mologous recombination(HR)is praised for the high accuracy.The MRE11-RAD50-NBS1(MRN)complex plays an important role in HR and is conserved across different species.The knowledge on the MRN complex mainly came from the previous studies in Saccharomyces cerevisiae and Caenorhabditis elegans,while studies in the last decades have revealed the role of mammalian MRN complex in DNA repair of higher animals.In this re-view,we first introduces the MRN complex regarding the composition,structure,and roles in HR.In addition,we discuss the human diseases such as ataxia-telangiectasia-like disorder,Nijmegen breakage syndrome,and Ni-jmegen breakage syndrome-like disorder that are caused by dysfunctions in the MRN complex.Furthermore,we summarize the mouse models established to study the clinical phenotypes of the above diseases.