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The MLL/SET family and haematopoiesis.
Biochimica et Biophysica Acta (BBA) - Gene Regulatory Mechanisms ( IF 4.7 ) Pub Date : 2020-05-07 , DOI: 10.1016/j.bbagrm.2020.194579
Eric T B Antunes 1 , Katrin Ottersbach 1
Affiliation  

As demonstrated through early work in Drosophila, members of the MLL/SET family play essential roles during embryonic development through their participation in large protein complexes that are central to epigenetic regulation of gene expression. One of its members, MLL1, has additionally received a lot of attention as it is a potent oncogenic driver in different types of leukaemia when aberrantly fused to a large variety of partners as a result of chromosomal translocations. Its exclusive association with cancers of the haematopoietic system has prompted a large number of investigations into the role of MLL/SET proteins in haematopoiesis, a summary of which was attempted in this review. Interestingly, MLL-rearranged leukaemias are particularly prominent in infant and paediatric leukaemia, which commonly initiate in utero. This, together with the known function of MLL/SET proteins in embryonic development, has focussed research efforts in recent years on understanding the role of this protein family in developmental haematopoiesis and how this may be subverted by MLL oncofusions in infant leukaemia. A detailed understanding of these prenatal events is essential for the development of new treatments that improve the survival specifically of this very young patient group.

中文翻译:

MLL / SET家族和造血功能。

正如果蝇早期工作所证明的那样,MLL / SET家族成员通过参与大型蛋白质复合体(在基因表达的后生调控中至关重要),在胚胎发育过程中发挥着重要作用。它的成员之一MLL1由于受到染色体易位异常融合到各种各样的伴侣中,是不同类型白血病的有效致癌驱动因子,因此受到了更多关注。它与造血系统癌症的排他性关联促使对MLL / SET蛋白在造血作用中的作用进行了大量研究,本综述对此进行了尝试。有趣的是,MLL重排的白血病在婴儿和小儿白血病中尤为突出,通常在子宫内引发。这个,连同MLL / SET蛋白在胚胎发育中的已知功能一起,近年来的研究重点集中在了解该蛋白家族在发育性造血中的作用以及如何在婴儿白血病中通过MLL肿瘤混和来颠覆这一点。对这些产前事件的详细了解对于开发新疗法至关重要,特别是可以改善这个非常年轻的患者群体的生存率。
更新日期:2020-05-07
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