Defective transcription-coupled repair of oxidative base damage in Cockayne syndrome patients from XP group G (Retracted Article. See vol 308, pg 1740, 2005)
Defective transcription-coupled repair of oxidative base damage in Cockayne syndrome patients from XP group G (Retracted Article. See vol 308, pg 1740, 2005)
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DOI：
10.1126/science.275.5302.990
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发表时间：
1997-02-14
期刊：
影响因子：
56.9
通讯作者：
Leadon, SA
中科院分区：
文献类型：
--
作者：
Cooper, PK;Nouspikel, T;Leadon, SA
在正常人类细胞中，由于紫外线引起的损伤优先通过转录偶联修复(TCR)过程中的核苷酸切除修复(NER)从活性基因中移除，该过程要求Cockayne综合征(CS)的基因产物存在缺陷。氧化损伤，包括胸腺嘧啶二醇，被证明可以通过TCR从正常人和患有NER缺陷的着色性干皮病(XP)-A、XP-F和XP-G患者的细胞中清除，但不能从患有严重CS的XP-G患者身上清除。因此，氧化损伤的TCR需要不同于其NER内切酶活性的XPG功能。这些结果增加了氧化损伤的TCR缺陷与CS相关的发育缺陷的可能性。
In normal human cells, damage due to ultraviolet light is preferentially removed from active genes by nucleotide excision repair (NER) in a transcription-coupled repair (TCR) process that requires the gene products defective in Cockayne syndrome (CS). Oxidative damage, including thymine glycols, is shown to be removed by TCR in cells from normal individuals and from xeroderma pigmentosum (XP)-A, XP-F, and XP-G patients who have NER defects but not from XP-G patients who have severe CS. Thus, TCR of oxidative damage requires an XPG function distinct from its NER endonuclease activity. These results raise the possibility that defective TCR of oxidative damage contributes to the developmental defects associated with CS.