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首页> 外文期刊>Molecular and Cellular Biology >LRP130, a Pentatricopeptide Motif Protein with a Noncanonical RNA-Binding Domain, Is Bound In Vivo to Mitochondrial and Nuclear RNAs
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LRP130, a Pentatricopeptide Motif Protein with a Noncanonical RNA-Binding Domain, Is Bound In Vivo to Mitochondrial and Nuclear RNAs

机译：LRP130，一种具有非规范RNA结合域的五肽基序蛋白，体内结合到线粒体和核RNA

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摘要

LRP130 (also known as LRPPRC) is an RNA-binding protein that is a constituent of postsplicing nuclear RNP complexes associated with mature mRNA. It belongs to a growing family of pentatricopeptide repeat (PPR) motif-containing proteins, several of which have been implicated in organellar RNA metabolism. We show here that only a fraction of LRP130 proteins are in nuclei and are directly bound in vivo to at least some of the same RNA molecules as the nucleocytoplasmic shuttle protein hnRNP A1. The majority of LRP130 proteins are located within mitochondria, where they are directly bound to polyadenylated RNAs in vivo. In vitro, LRP130 binds preferentially to polypyrimidines. This RNA-binding activity maps to a domain in its C-terminal region that does not contain any previously described RNA-binding motifs and that contains only 2 of the 11 predicted PPR motifs. Therefore, LRP130 is a novel type of RNA-binding protein that associates with both nuclear and mitochondrial mRNAs and as such is a potential candidate for coordinating nuclear and mitochondrial gene expression. These findings provide the first identification of a mammalian protein directly bound to mitochondrial RNA in vivo and provide a possible molecular explanation for the recently described association of mutations in LRP130 with cytochrome c oxidase deficiency in humans.
机译：LRP130（也称为LRPPRC）是一种RNA结合蛋白，是与成熟mRNA相关的后核RNP复合物的组成部分。它属于一个不断增长的含有五肽重复序列（PPR）基序的蛋白质家族，其中一些与细胞器RNA代谢有关。我们在这里显示，只有一小部分LRP130蛋白位于细胞核中，并且在体内直接与至少一些与核质穿梭蛋白hnRNP A1相同的RNA分子结合。大多数LRP130蛋白位于线粒体内，在体内它们直接与聚腺苷酸RNA结合。在体外，LRP130优先与聚嘧啶结合。这种RNA结合活性映射到其C端区域中的一个域，该域不包含任何先前描述的RNA结合基序，并且仅包含11个预测的PPR基序中的两个。因此，LRP130是一种新型的RNA结合蛋白，与核和线粒体mRNA都相关，因此是协调核和线粒体基因表达的潜在候选者。这些发现为体内首次直接结合线粒体RNA的哺乳动物蛋白提供了首次鉴定，并为最近描述的LRP130突变与人类细胞色素 c 氧化酶缺乏的关联提供了可能的分子解释。

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