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GRSF1 regulates RNA processing in mitochondrial RNA granules

机译：GRSF1调节线粒体RNA颗粒中的RNA加工

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摘要

Various specialized domains have been described in the cytosol and the nucleus; however, little is known about compartmentalization within the mitochondrial matrix. GRSF1 (G-rich sequence factor 1) is an RNA binding protein that was previously reported to localize in the cytosol. We found that an isoform of GRSF1 accumulates in discrete foci in the mitochondrial matrix. These foci are composed of nascent mitochondrial RNA and also contain RNase P, an enzyme that participates in mitochondrial RNA processing. GRSF1 was found to interact with RNase P and to be required for processing of both classical and tRNA-less RNA precursors. In its absence, cleavage of primary RNA transcripts is abnormal, leading to decreased expression of mitochondrially encoded proteins and mitochondrial dysfunction. Our findings suggest that the foci containing GRSF1 and RNase P correspond to sites where primary RNA transcripts converge to be processed. We have termed these large ribonucleoprotein structures "mitochondrial RNA granules."
机译：在细胞质和细胞核中已经描述了各种专门的结构域。然而，关于线粒体基质内的区室化了解甚少。 GRSF1（富含G的序列因子1）是一种RNA结合蛋白，以前被报道位于细胞质中。我们发现GRSF1的同工型积累在线粒体矩阵中的离散焦点中。这些病灶由新生的线粒体RNA组成，还包含RNase P（一种参与线粒体RNA加工的酶）。发现GRSF1与RNase P相互作用，是加工经典和无tRNA的RNA前体所必需的。在没有它的情况下，一级RNA转录物的切割是异常的，从而导致线粒体编码蛋白的表达降低和线粒体功能障碍。我们的研究结果表明，包含GRSF1和RNase P的病灶对应于一级RNA转录本聚合加工的位点。我们将这些大的核糖核蛋白结构称为“线粒体RNA颗粒”。

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