The Bloom syndrome complex is a DNA damage repair machine. It consists of several protein components which are functional in isolation, but interdependent in cells for the maintenance of accurate homologous recombination. Mutations to any of the genes encoding these proteins cause numerous physical and developmental markers as well as phenotypes of genome instability, infertility, and cancer predisposition. Here we review the published structural and biochemical data on each of the components of the complex: the helicase BLM, the type IA topoisomerase TOP3A, and the OB-fold-containing RMI and RPA subunits. We describe how each component contributes to function, interacts with each other, and the DNA that it manipulates/repairs.

布卢姆综合征复合体是一种 DNA 损伤修复机器。它由几种蛋白质成分组成，这些成分单独发挥作用，但在细胞中相互依赖，以维持准确的同源重组。编码这些蛋白质的任何基因的突变会导致许多物理和发育标记以及基因组不稳定、不育和癌症易感性的表型。在这里，我们回顾了关于复合物每个组件的已发表的结构和生化数据：解旋酶 BLM、IA 型拓扑异构酶 TOP3A 以及包含 OB 折叠的 RMI 和 RPA 亚基。我们描述了每个组件如何促进功能、相互作用以及它操纵/修复的 DNA。