CSB is a component of RNA pol I transcription
CSB is a component of RNA pol I transcription
复制标题
DOI：
10.1016/s1097-2765(02)00678-0
复制
发表时间：
2002-10-01
期刊：
影响因子：
16
通讯作者：
Egly, JM
中科院分区：
文献类型：
--
作者：
Bradsher, J;Auriol, J;Egly, JM
CSB基因突变导致人类Cockayne综合征（CS）。在这里，我们提供的证据表明，CSB不仅在核质中，而且在核仁内的一个复杂的（CSB IP/150），其中包含RNA聚合酶I，TFIIH，和XPG，并促进有效的rRNA合成。CSB在体外RNA pol I转录中具有活性，并且在CSB缺陷细胞中转染时恢复rRNA合成。我们还表明，CSB中的突变，以及在XPB和XPD基因，所有这些赋予CS，干扰CSB IP/150内的RNA聚合酶I/TFIIH相互作用。除了揭示CSB在rRNA合成中的意想不到的功能外，我们还表明这种复合物的脆弱性可能是导致CS表型的一个因素。
Mutation in the CSB gene results in the human Cockayne's syndrome (CS). Here, we provide evidence that CSB is found not only in the nucleoplasm but also in the nucleolus within a complex (CSB IP/150) that contains RNA pol I, TFIIH, and XPG and promotes efficient rRNA synthesis. CSB is active in in vitro RNA pol I transcription and restores rRNA synthesis when transfected in CSB-deficient cells. We also show that mutations in CSB, as well as in XPB and XPD genes, all of which confer CS, disturb the RNA pol I/TFIIH interaction within the CSB IP/150. In addition to revealing an unanticipated function for CSB in rRNA synthesis, we show that the fragility of this complex could be one factor contributing to the CS phenotype.