科凯恩综合征B组蛋白增强RNA聚合酶II的延伸作用。
Cockayne syndrome group B protein enhances elongation by RNA polymerase II.
作者信息
Selby C P, Sancar A
机构信息
Department of Biochemistry and Biophysics, University of North Carolina School of Medicine, Chapel Hill, NC 27599-7260, USA.
出版信息
Proc Natl Acad Sci U S A. 1997 Oct 14;94(21):11205-9. doi: 10.1073/pnas.94.21.11205.
Abstract
Cockayne syndrome (CS) is characterized by impaired physical and mental development. Two complementation groups, CSA and CSB, have been identified. Here we report that the CSB gene product enhances elongation by RNA polymerase II. CSB stimulated the rate of elongation on an undamaged template by a factor of about 3. A thymine-thymine cyclobutane dimer located in the template strand is known to be a strong block to transcription. Addition of CSB to the blocked polymerase resulted in addition of one nucleotide to the nascent transcript. Finally, addition of transcription factor IIS is known to cause polymerase blocked at a thymine-thymine cyclobutane dimer to digest its nascent transcript, and CSB counteracted this transcript shortening action of transcription factor IIS. Thus a deficiency in transcription elongation may contribute to the CS phenotype.
摘要
科凯恩综合征（CS）的特征是身体和智力发育受损。已经鉴定出两个互补组，即CSA和CSB。在此我们报告，CSB基因产物可增强RNA聚合酶II的延伸能力。CSB将未受损模板上的延伸速率提高了约3倍。已知位于模板链中的胸腺嘧啶-胸腺嘧啶环丁烷二聚体是转录的强大阻碍。向受阻的聚合酶中添加CSB会导致新生转录本添加一个核苷酸。最后，已知添加转录因子IIS会使在胸腺嘧啶-胸腺嘧啶环丁烷二聚体处受阻的聚合酶消化其新生转录本，而CSB可抵消转录因子IIS的这种转录本缩短作用。因此，转录延伸缺陷可能导致CS表型。
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1
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Proc Natl Acad Sci U S A. 1997 Apr 29;94(9):4306-11. doi: 10.1073/pnas.94.9.4306.
2
Defective transcription-coupled repair of oxidative base damage in Cockayne syndrome patients from XP group G.XP组G型科凯恩综合征患者中氧化性碱基损伤的转录偶联修复缺陷。
Science. 1997 Feb 14;275(5302):990-3. doi: 10.1126/science.275.5302.990.
3
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Nucleic Acids Res. 1997 Feb 15;25(4):787-93. doi: 10.1093/nar/25.4.787.
4
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J Biol Chem. 1997 Jan 17;272(3):1885-90. doi: 10.1074/jbc.272.3.1885.
5
The RNA polymerase II general elongation factors.RNA聚合酶II通用延伸因子。
Trends Biochem Sci. 1996 Sep;21(9):351-5.
6
Cockayne syndrome--a primary defect in DNA repair, transcription, both or neither?科凯恩综合征——DNA修复、转录的原发性缺陷，还是两者皆有缺陷或两者均无缺陷？
Bioessays. 1996 Sep;18(9):731-8. doi: 10.1002/bies.950180908.
7
DNA excision repair.DNA切除修复
Annu Rev Biochem. 1996;65:43-81. doi: 10.1146/annurev.bi.65.070196.000355.
8
Interactions involving the human RNA polymerase II transcription/nucleotide excision repair complex TFIIH, the nucleotide excision repair protein XPG, and Cockayne syndrome group B (CSB) protein.涉及人类RNA聚合酶II转录/核苷酸切除修复复合物TFIIH、核苷酸切除修复蛋白XPG和科凯恩综合征B组（CSB）蛋白的相互作用。
Biochemistry. 1996 Feb 20;35(7):2157-67. doi: 10.1021/bi9524124.
9
Double mutants of Saccharomyces cerevisiae with alterations in global genome and transcription-coupled repair.酿酒酵母在全基因组和转录偶联修复方面发生改变的双突变体。
Mol Cell Biol. 1996 Feb;16(2):496-502. doi: 10.1128/MCB.16.2.496.
10
DNA repair. Engagement with transcription.DNA修复。与转录的关联。
Nature. 1993 May 13;363(6425):114-5. doi: 10.1038/363114a0.
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