The MRE11 complex: starting from the ends

Abstract

The MRE11 complex, consisting of meiotic recombination 11 (MRE11), RAD50 and Nijmegen breakage syndrome 1 (NBS1; also known as nibrin), is of key importance in the DNA damage response to DNA double-strand breaks, and therefore regulates genome stability. The MRE11 complex regulates repair of DNA double-strand breaks in several contexts, including replication, telomere homeostasis, meiosis, apoptosis and immune system development. Structural studies of the MRE11 complex have provided insights into the distinct roles that different components in this complex have. The globular domain of the MRE11 complex is important for DNA binding, the RAD50 hook domain mediates complex homodimerization and NBS1 mediates regulation of the complex. Mouse models for different alleles of the MRE11 complex have been developed on the basis of these structural studies, and show clinical features of the human syndromes that the MRE11 complex has been linked to.