Bijvoet Center for Biomolecular Research, Utrecht University, Padualaan 8, 3584 CH Utrecht, The Netherlands.
Molecules. 2018 Dec 5;23(12):3205. doi: 10.3390/molecules23123205.
Numerous proteins are involved in the multiple pathways of the DNA damage response network and play a key role to protect the genome from the wide variety of damages that can occur to DNA. An example of this is the structure-specific endonuclease ERCC1-XPF. This heterodimeric complex is in particular involved in nucleotide excision repair (NER), but also in double strand break repair and interstrand cross-link repair pathways. Here we review the function of ERCC1-XPF in various DNA repair pathways and discuss human disorders associated with ERCC1-XPF deficiency. We also overview our molecular and structural understanding of XPF-ERCC1.
许多蛋白质参与 DNA 损伤反应网络的多个途径，并在保护基因组免受可能发生在 DNA 上的各种损伤方面发挥关键作用。结构特异性内切酶 ERCC1-XPF 就是一个例子。该异源二聚体复合物特别参与核苷酸切除修复 (NER)，但也参与双链断裂修复和链间交联修复途径。本文综述了 ERCC1-XPF 在各种 DNA 修复途径中的功能，并讨论了与 ERCC1-XPF 缺陷相关的人类疾病。我们还概述了我们对 XPF-ERCC1 的分子和结构的理解。
