Regulation of the Fanconi anemia pathway by monoubiquitination
Regulation of the Fanconi anemia pathway by monoubiquitination
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DOI：
10.1016/s1044-579x(02)00102-5
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发表时间：
2003-02-01
影响因子：
14.5
通讯作者：
D'Andrea, AD
中科院分区：
文献类型：
--
作者：
Gregory, RC;Taniguchi, T;D'Andrea, AD
范可尼贫血（FA）是一种常染色体隐性遗传癌症易感性综合征，以多种先天性异常为特征。骨髓衰竭和细胞对丝裂霉素 C (MMC) 的敏感性。迄今为止，六个 FA 基因已被克隆，其编码的蛋白质以新的途径发挥作用。 FA 途径是细胞对 DNA 损伤做出正常反应所必需的。 DNA 损伤后。该途径被激活，导致 FA 蛋白单泛素化。 FANCD2，及其针对亚核病灶的靶向。 FA 途径的破坏会导致 FANCD2 核灶的缺失，从而导致 FA 的细胞和临床异常。在这里，我们回顾了最近描述 FANCD2 蛋白受调节的单泛素化的研究，并讨论了 FA 途径与其他 DNA 损伤反应途径的相互作用。 (C) 2002 Elsevier Science Ltd. 保留所有权利。
Fanconi anemia (FA) is an autosomal recessive cancer susceptibility syndrome characterized by multiple congenital anomalies. bone marrow failure, and cellular sensitivity to mitomycin C (MMC). To date, six FA genes have been cloned, and the encoded proteins function in a novel pathway. The FA pathway is required for the normal cellular response to DNA damage. Following DNA damage. the pathway is activated, leading to monoubiquitination of the FA protein. FANCD2, and its targeting to subnuclear foci. Disruption of the FA pathway results in the absence of FANCD2 nuclear foci, leading to the cellular and clinical abnormalities of FA. Here, we review the recent studies describing the regulated monoubiquitination of the FANCD2 protein and discuss the interaction of the FA pathway with other DNA damage response pathways. (C) 2002 Elsevier Science Ltd. All rights reserved.