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首页> 外文期刊>International Journal of Molecular Sciences >Deubiquitinase MYSM1 in the Hematopoietic System and beyond: A Current Review
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Deubiquitinase MYSM1 in the Hematopoietic System and beyond: A Current Review

机译：脱血素酶Mysm1在造血系统及以后：目前的评论

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摘要

MYSM1 has emerged as an important regulator of hematopoietic stem cell function, blood cell production, immune response, and other aspects of mammalian physiology. It is a metalloprotease family protein with deubiquitinase catalytic activity, as well as SANT and SWIRM domains. MYSM1 normally localizes to the nucleus, where it can interact with chromatin and regulate gene expression, through deubiquitination of histone H2A and non-catalytic contacts with other transcriptional regulators. A cytosolic form of MYSM1 protein was also recently described and demonstrated to regulate signal transduction pathways of innate immunity, by promoting the deubiquitination of TRAF3, TRAF6, and RIP2. In this work we review the current knowledge on the molecular mechanisms of action of MYSM1 protein in transcriptional regulation, signal transduction, and potentially other cellular processes. The functions of MYSM1 in different cell types and aspects of mammalian physiology are also reviewed, highlighting the key checkpoints in hematopoiesis, immunity, and beyond regulated by MYSM1. Importantly, mutations in MYSM1 in human were recently linked to a rare hereditary disorder characterized by leukopenia, anemia, and other hematopoietic and developmental abnormalities. Our growing knowledge of MYSM1 functions and mechanisms of actions sheds important insights into its role in mammalian physiology and the etiology of the MYSM1-deficiency disorder in human.
机译：Mysm1已成为造血干细胞功能，血细胞生产，免疫应答等哺乳动物生理学的其他方面的重要调节因素。它是一种金属蛋白酶蛋白质蛋白质，具有脱硫酶催化活性，以及​​Sant和晶体结构域。 Mysm1通常通过与其他转录调节剂的非催化接触和其他转录调节剂的非催化接触来定位于细胞核，在那里它可以与染色质相互作用并调节基因表达。最近还通过促进TRAF3，TRAF6和RIP2的脱氮来调节先天免疫信号转导途径的胞质体形式的Mysm1蛋白。在这项工作中，我们审查了目前关于Mysm1蛋白在转录调节，信号转导和可能其他细胞过程中的Mysm1蛋白的分子机制的知识。还审查了MySM1在不同细胞类型和哺乳动物生理学的方面的功能，突出了造血，免疫力，免疫力和超越Mysm1的关键检查点。重要的是，人类中的Mysm1中的突变最近与稀有的遗传性疾病相关，其特征是白细胞，贫血和其他造血和发育异常。我们对Mysm1功能的越来越多的知识和行动机制揭示了其在哺乳动物生理学中的作用以及人类中Mysm1缺乏症的病因的重要性见解。

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