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首页> 外文期刊>Molecular and Cellular Biology >Definition of a Short Region of XPG Necessary for TFIIH Interaction and Stable Recruitment to Sites of UV Damage
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Definition of a Short Region of XPG Necessary for TFIIH Interaction and Stable Recruitment to Sites of UV Damage

机译：定义TFIIH相互作用和稳定招募紫外线损伤位点所需的XPG短区域

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摘要

XPG is the human endonuclease that cuts 3′ to DNA lesions during nucleotide excision repair. Missense mutations in XPG can lead to xeroderma pigmentosum (XP), whereas truncated or unstable XPG proteins cause Cockayne syndrome (CS), normally yielding life spans of <7 years. One XP-G individual who had advanced XP/CS symptoms at 28 years has been identified. The genetic, biochemical, and cellular defects in this remarkable case provide insight into the onset of XP and CS, and they reveal a previously unrecognized property of XPG. Both of this individual's XPG alleles produce a severely truncated protein, but an infrequent alternative splice generates an XPG protein lacking seven internal amino acids, which can account for his very slight cellular UV resistance. Deletion of XPG amino acids 225 to 231 does not abolish structure-specific endonuclease activity. Instead, this region is essential for interaction with TFIIH and for the stable recruitment of XPG to sites of local UV damage after the prior recruitment of TFIIH. These results define a new functional domain of XPG, and they demonstrate that recruitment of DNA repair proteins to sites of damage does not necessarily lead to productive repair reactions. This observation has potential implications that extend beyond nucleotide excision repair.
机译：XPG是一种人类核酸内切酶，可在核苷酸切除修复过程中将3'切割为DNA损伤。 XPG中的错义突变可以导致色素干皮病（XP），而截短的或不稳定的XPG蛋白会导致Cockayne综合征（CS），正常情况下寿命不到7年。已鉴定出一名在28岁时出现XP / CS症状的XP-G个人。在这种非凡的情况下，遗传，生物化学和细胞缺陷为XP和CS的发病提供了见识，并且它们揭示了XPG以前无法识别的特性。此人的两个 XPG 等位基因均会产生严重截短的蛋白质，但很少的替代剪接会产生缺少七个内部氨基酸的XPG蛋白质，这可能解释了他对细胞的轻微紫外线抵抗力。 XPG氨基酸225至231的删除不会消除结构特异性核酸内切酶的活性。相反，该区域对于与TFIIH交互作用以及在TFIIH先前募集后稳定地将XPG募集到局部UV损伤位点至关重要。这些结果定义了XPG的新功能域，并且它们表明将DNA修复蛋白募集到损伤位点不一定导致有效的修复反应。该观察结果具有潜在的意义，其范围超出了核苷酸切除修复。

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