Regulator of ribosome synthesis 1(RRS1) was first discovered in yeast in 1999. In yeast, RRS1 is a multifunctional protein. RRS1 and ribosome production factor 2 are required for the maturation of 25S rRNA and the production of the 60S ribosomal subunit. In mammals, RRS1 contributes to chromosome congression and telo⁃meres cluster. RRS1 is also involved in endoplasmic reticulum stress response in Huntington disease. Here, we sys⁃tematically reviewed the function of RRS1 and its relationship with diseases.%1999年,Tsuno等在酵母中发现了一个核糖体合成调控蛋白,命名为RRS1。在酵母中,RRS1是一个多功能的蛋白,与核糖体产物因子2相互作用,在25S rRNA成熟过程中起重要作用,并参与核糖体60S大亚基的生物合成。在哺乳动物细胞中,RRS1参与细胞周期中染色体在赤道板的聚集和端粒的聚集。RRS1在亨廷顿病等的发生中发挥着重要作用。我们简要概述RRS1的功能及其与疾病的关系。
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