How SUMOylation Fine-Tunes the Fanconi Anemia DNA Repair Pathway.
How SUMOylation Fine-Tunes the Fanconi Anemia DNA Repair Pathway.
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SUMOylation 如何微调范可尼贫血 DNA 修复途径。
DOI：
10.3389/fgene.2016.00061
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发表时间：
2016
影响因子：
3.7
通讯作者：
Huang TT
中科院分区：
文献类型：
--
作者：
Coleman KE;Huang TT
范可尼贫血（FA）是一种罕见的人类遗传性疾病，其特征是发育缺陷，骨髓衰竭和癌症易感性，主要是由于DNA链间交联（ICL）修复缺陷。通过FA DNA修复途径的ICL修复是一个复杂的多步骤过程，涉及至少19种FANC蛋白和多种DNA修复活性的协调，包括同源重组、核苷酸切除修复和跨损伤合成（TLS）。SUMO化是几种DNA修复途径的关键调节因子，然而，这种修饰在控制FA途径中的作用知之甚少。在这里，我们总结了最近的进展微调FA通路的小泛素样修饰剂（SUMO）靶向的泛素连接酶（STUBLs）和其他SUMO相关的相互作用，并讨论了这些发现的影响，在设计新的治疗方法，以减轻FA相关的条件，包括癌症。
Fanconi anemia (FA) is a rare human genetic disorder characterized by developmental defects, bone marrow failure and cancer predisposition, primarily due to a deficiency in the repair of DNA interstrand crosslinks (ICLs). ICL repair through the FA DNA repair pathway is a complicated multi-step process, involving at least 19 FANC proteins and coordination of multiple DNA repair activities, including homologous recombination, nucleotide excision repair and translesion synthesis (TLS). SUMOylation is a critical regulator of several DNA repair pathways, however, the role of this modification in controlling the FA pathway is poorly understood. Here, we summarize recent advances in the fine-tuning of the FA pathway by small ubiquitin-like modifier (SUMO)-targeted ubiquitin ligases (STUbLs) and other SUMO-related interactions, and discuss the implications of these findings in the design of novel therapeutics for alleviating FA-associated condition, including cancer.