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范可尼贫血蛋白的3R协调作用。

3R coordination by Fanconi anemia proteins.

作者信息

Macé Gaëtane, Bogliolo Massimo, Guervilly Jean-Hugues, Dugas du Villard Jean Antoine, Rosselli Filippo

机构信息

Institut Gustave-Roussy PR2, UPR2169 du CNRS, 39, rue Camille-Desmoulins, 94805 Villejuif cedex, France.

出版信息

Biochimie. 2005 Jul;87(7):647-58. doi: 10.1016/j.biochi.2005.05.003.

Abstract

Fanconi anemia (FA) is a recessive cancer prone syndrome featuring bone marrow failure and hypersensitivity to DNA crosslinks. Nine FA genes have been isolated so far. The biochemical function(s) of the FA proteins remain(s) poorly determined. However, a large consensus exists on the evidence that, to cope with DNA cross-links, a cell needs a functional FA pathway. In this review, we resume current understanding of how the FA pathway works in response to DNA damage and how it is integrated in a complex network of proteins involved in the maintenance of the genetic stability.

摘要

范可尼贫血(FA)是一种隐性的易患癌症综合征,其特征为骨髓衰竭和对DNA交联超敏。到目前为止,已分离出9个FA基因。FA蛋白的生化功能仍未明确。然而,有大量证据表明,细胞应对DNA交联需要一个功能性的FA通路,这一点已达成广泛共识。在本综述中,我们总结了目前对FA通路如何响应DNA损伤发挥作用,以及如何整合到维持遗传稳定性的复杂蛋白质网络中的理解。

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