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由范可尼贫血蛋白和乳腺癌易感基因(BRCA)蛋白组成的DNA损伤反应网络的出现。

Emergence of a DNA-damage response network consisting of Fanconi anaemia and BRCA proteins.

作者信息

Wang Weidong

机构信息

Laboratory of Genetics, National Institute on Aging, National Institutes of Health, 333 Cassell drive, Baltimore, Maryland 21093, USA.

出版信息

Nat Rev Genet. 2007 Oct;8(10):735-48. doi: 10.1038/nrg2159. Epub 2007 Sep 4.

Abstract

Fanconi anaemia (FA) has recently become an attractive model to study breast cancer susceptibility (BRCA) genes, as three FA genes, FANCD1, FANCN and FANCJ, are identical to the BRCA genes BRCA2, PALB2 and BRIP1. Increasing evidence shows that FA proteins function as signal transducers and DNA-processing molecules in a DNA-damage response network. This network consists of many proteins that maintain genome integrity, including ataxia telangiectasia and Rad3 related protein (ATR), Bloom syndrome protein (BLM), and BRCA1. Now that the gene that is defective in the thirteenth and last assigned FA complementation group (FANCI) has been identified, I discuss what is known about FA proteins and their interactive network, and what remains to be discovered.

摘要

范可尼贫血(FA)最近成为研究乳腺癌易感性(BRCA)基因的一个有吸引力的模型,因为三个FA基因,FANCD1、FANCN和FANCJ,分别与BRCA基因BRCA2、PALB2和BRIP1相同。越来越多的证据表明,FA蛋白在DNA损伤反应网络中作为信号转导分子和DNA加工分子发挥作用。这个网络由许多维持基因组完整性的蛋白质组成,包括共济失调毛细血管扩张症和Rad3相关蛋白(ATR)、布卢姆综合征蛋白(BLM)以及BRCA1。鉴于在已确定的第十三个也是最后一个FA互补组(FANCI)中存在缺陷的基因已被识别出来,我将讨论关于FA蛋白及其相互作用网络的已知信息,以及有待发现的内容。

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