Institute of Molecular Medicine I, Medical Faculty, Heinrich Heine University Düsseldorf, 40225 Düsseldorf, Germany.
Institute of Experimental Internal Medicine, Otto von Guericke University, 39120 Magdeburg, Germany.
Int J Mol Sci. 2023 Oct 25;24(21):15552. doi: 10.3390/ijms242115552.
The survival motor neuron (SMN) complex is a multi-megadalton complex involved in post-transcriptional gene expression in eukaryotes via promotion of the biogenesis of uridine-rich small nuclear ribonucleoproteins (UsnRNPs). The functional center of the complex is formed from the SMN/Gemin2 subunit. By binding the pentameric ring made up of the Sm proteins SmD1/D2/E/F/G and allowing for their transfer to a uridine-rich short nuclear RNA (UsnRNA), the Gemin2 protein in particular is crucial for the selectivity of the Sm core assembly. It is well established that post-translational modifications control UsnRNP biogenesis. In our work presented here, we emphasize the crucial role of Gemin2, showing that the phospho-status of Gemin2 influences the capacity of the SMN complex to condense in Cajal bodies (CBs) in vivo. Additionally, we define Gemin2 as a novel and particular binding partner and phosphorylation substrate of the mTOR pathway kinase ribosomal protein S6 kinase beta-1 (p70S6K). Experiments using size exclusion chromatography further demonstrated that the Gemin2 protein functions as a connecting element between the 6S complex and the SMN complex. As a result, p70S6K knockdown lowered the number of CBs, which in turn inhibited in vivo UsnRNP synthesis. In summary, these findings reveal a unique regulatory mechanism of UsnRNP biogenesis.
运动神经元存活(SMN)复合物是一个涉及真核生物转录后基因表达的兆道尔顿复合物,通过促进富含尿嘧啶的小核核糖核蛋白(UsnRNPs)的生物发生。复合物的功能中心由 SMN/Gemin2 亚基形成。通过与由 Sm 蛋白 SmD1/D2/E/F/G 组成的五聚体环结合并允许它们转移到富含尿嘧啶的短核 RNA(UsnRNA)上,Gemin2 蛋白对于 Sm 核心组装的选择性至关重要。已经证实,翻译后修饰控制着 UsnRNP 的生物发生。在我们这里提出的工作中,我们强调了 Gemin2 的关键作用,表明 Gemin2 的磷酸化状态影响 SMN 复合物在体内 Cajal 体(CBs)中浓缩的能力。此外,我们将 Gemin2 定义为 mTOR 途径激酶核糖体蛋白 S6 激酶β-1(p70S6K)的新型和特殊结合伴侣和磷酸化底物。使用排阻色谱的实验进一步表明,Gemin2 蛋白作为 6S 复合物和 SMN 复合物之间的连接元件发挥作用。结果,p70S6K 敲低降低了 CB 的数量,这反过来又抑制了体内 UsnRNP 的合成。总之,这些发现揭示了 UsnRNP 生物发生的独特调节机制。