The seven xeroderma pigmentosum proteins (XPps), XPA–XPG, coordinate the nucleotide excision repair (NER) pathway, promoting the excision of DNA lesions caused by exposition to ionizing radiation, majorly from ultraviolet light. Significant efforts are made to investigate NER since mutations in any of the seven XPps may cause the xeroderma pigmentosum and trichothiodystrophy diseases. However, these proteins collaborate with other pivotal players in all known NER steps to accurately exert their purposes. Therefore, in the old and ever-evolving field of DNA repair, it is imperative to reexamine and describe their structures to understand NER properly. This work provides an up-to-date review of the protein structural aspects of the closest partners that directly interact and influence XPps: RAD23B, CETN2, DDB1, RPA (RPA70, 32, and 14), p8 (GTF2H5), and ERCC1. Structurally and functionally vital domains, regions, and critical residues are reexamined, providing structural lessons and perspectives about these indispensable proteins in the NER and other DNA repair pathways. By gathering all data related to the major human xeroderma pigmentosum-interacting proteins, this review will aid newcomers on the subject and guide structural and functional future studies.

中文翻译：

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每个主角都有一个伙伴：核苷酸切除修复中人类色素性干皮病结合蛋白的结构方面

七种色素性干皮病蛋白 (XPps)，XPA-XPG，协调核苷酸切除修复 (NER) 途径，促进切除因暴露于电离辐射（主要是紫外线）引起的 DNA 损伤。由于 7 个 XPps 中任何一个的突变都可能导致色素性干皮病和毛发硫营养不良疾病，因此为研究 NER 做出了重大努力。然而，这些蛋白质在所有已知的 NER 步骤中与其他关键参与者合作，以准确发挥其目的。因此，在古老且不断发展的 DNA 修复领域，必须重新检查和描述其结构以正确理解 NER。这项工作提供了对直接相互作用和影响 XPps 的最亲密伙伴的蛋白质结构方面的最新回顾：RAD23B、CETN2、DDB1、RPA（RPA70、32 和 14）、p8（GTF2H5）、和ERCC1。重新检查结构和功能上至关重要的域、区域和关键残基，提供有关 NER 和其他 DNA 修复途径中这些不可或缺的蛋白质的结构教训和观点。通过收集与主要人类色素性干皮病相互作用蛋白相关的所有数据，本综述将有助于该主题的新手，并指导未来的结构和功能研究。