Cockayne syndrome (CS) is characterized by progressive multisystem degeneration and is classified as a segmental premature aging syndrome. The majority of CS cases are caused by defects in the CS complementation group B (CSB) protein and the rest are mainly caused by defects in the CS complementation group A (CSA) protein. Cells from CS patients are sensitive to UV light and a number of other DNA damaging agents including various types of oxidative stress. The cells also display transcription deficiencies, abnormal apoptotic response to DNA damage, and DNA repair deficiencies. Herein we have critically reviewed the current knowledge about known protein interactions of the CS proteins. The review focuses on the participation of the CSB and CSA proteins in many different protein interactions and complexes, and how these interactions inform us about pathways that are defective in the disease.

中文翻译：

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Cockayne 综合征蛋白的多个相互作用伙伴：对基因组和转录组维护的影响。

Cockayne 综合征 (CS) 的特征是进行性多系统变性，被归类为节段性早衰综合征。大多数 CS 病例是由 CS 互补组 B (CSB) 蛋白的缺陷引起的，其余的主要是由 CS 互补组 A (CSA) 蛋白的缺陷引起的。CS 患者的细胞对紫外线和许多其他 DNA 损伤剂（包括各种类型的氧化应激）敏感。这些细胞还表现出转录缺陷、对 DNA 损伤的异常凋亡反应和 DNA 修复缺陷。在此，我们批判性地回顾了有关 CS 蛋白已知蛋白质相互作用的当前知识。该评论侧重于 CSB 和 CSA 蛋白在许多不同的蛋白质相互作用和复合物中的参与，