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泉综合征蛋白USP7通过BCOR-ncPRC1.1控制神经元分化

Joyce Wolf van der Meer¹, Axelle Larue², Jan A van der Knaap¹

¹Department of Biochemistry, Erasmus University Medical Center, 3025 GD Rotterdam, The Netherlands.

Genes & development

|February 7, 2025

概括

乌比奎丁特异性蛋白酶7 (USP7) 对于神经发育至关重要,通过BCOR-ncPRC1.1复合体调节基因表达. 这一发现揭示了神经发育障碍中共享的表观遗传网络.

科学领域:

表观遗传学在表观遗传学中,表观遗传学是指表观遗传学.
神经生物学神经生物学神经生物学
分子生物学分子生物学

背景情况:

泛素特异蛋白酶7 (USP7) 基因中的致病变体与神经发育障碍的Hao-Fountain综合征有关.
在神经发育中USP7的确切功能在很大程度上仍未被定义.

研究的目的:

阐明神经元分化过程中USP7的调节电路.
在神经发育的背景下,确定USP7的关键效应因子和向基因.

主要方法:

量化蛋白质组学,转录组学和表观组学被用来绘制USP7的监管网络.
在神经元分化模型 (胚胎干细胞和SH-SY5Y细胞) 中进行了功能丧失实验.

主要成果:

USP7活动对于驱动神经干细胞分化和神经元分化的转录程序至关重要.
USP7调节了Polycomb抑制复合体1 (ncPRC1.1和ncPRC1.6) 组件的剂量,特别影响H2AK119ub1.1.
鉴定出BCOR-ncPRC1.1,但不是ncPRC1.6,是USP7在神经元分化和基因调节中的作用中介的关键效应因子.

结论:

在神经元分化过程中,USP7通过BCOR-ncPRC1.1复合体控制了很大一部分基因调节.
涉及USP7和ncPRC1.1的共享表观遗传网络的失调可能是相关神经发育障碍的基础.

关键词:

泉综合征泉综合征聚合的多元.USP7 USP7 美国在这种情况下,染色染色素神经发育的神经发育转录转录是一种转录.在任何地方都是无处不在的.

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