WRN蛋白与Werner综合征。

Jianyuan Luo
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引用次数: 7

摘要

沃纳综合征是一种常染色体隐性遗传病,与早衰和癌症易感性相关。来自维尔纳综合征患者的细胞表现出增加的基因组不稳定性,并且对DNA损伤剂极度敏感。Werner综合征是由WRN基因突变引起的。WRN蛋白是RecQ DNA解旋酶家族的成员。它不仅像RecQ家族的其他成员一样包含一个保守的3'-5'解旋酶结构域,而且包含一个独特的3'-5'外切酶结构域。WRN识别特定的DNA结构作为底物,是DNA代谢的中间体。WRN与许多其他蛋白质相互作用,这些蛋白质通过不同的途径在端粒维持、DNA复制和DNA修复中发挥作用。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
WRN protein and Werner syndrome.

Werner syndrome is an autosomal recessive disorder associated with premature aging and cancer predisposition. Cells from Werner syndrome patients show increased genomic instability and are hypersensitive to DNA damage agents. Werner syndrome is caused by mutations of the WRN gene. WRN protein is a member of RecQ DNA helicase family. It not only contains a conserved 3'-5' helicase domain as other members of the RecQ family but also contains a unique 3'-5' exonuclease domain. WRN recognizes specific DNA structures as substrates which are intermediates of DNA metabolism. WRN interacts with many other proteins, which function in telomere maintenance, DNA replication, and DNA repair through different pathways.

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