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Matrin3: connecting gene expression with the nuclear matrix

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As indicated by its name, Matrin3 was discovered as a component of the nuclear matrix, an insoluble fibrogranular network that structurally organizes the nucleus. Matrin3 possesses both DNA‐ and RNA‐binding domains and, consistent with this, has been shown to function at a number of stages in the life cycle of messenger RNAs. These numerous activities indicate that Matrin3, and indeed the nuclear matrix, do not just provide a structural framework for nuclear activities but also play direct functional roles in these activities. Here, we review the structure, functions, and molecular interactions of Matrin3 and of Matrin3‐related proteins, and the pathologies that can arise upon mutation of Matrin3. WIREs RNA 2016, 7:303–315. doi: 10.1002/wrna.1336 This article is categorized under: RNA Interactions with Proteins and Other Molecules > Protein–RNA Interactions: Functional Implications RNA Processing > Splicing Regulation/Alternative Splicing RNA in Disease and Development > RNA in Disease
Domain organization of Matrin3 and related proteins. (a) Schematic diagram of structural organization of Matrin3 (middle), ZNF638 (top), and RBM20 (bottom). The percent sequence identity between the RNA recognition motif (RRM) and zinc‐finger (ZF) domains of Matrin3 compared with ZNF638 and RBM20 is indicated. Disease‐associated amino acid residues S85, F115, P154, and T622 and the phosphorylation sites at S188 and S208 are also indicated. (b) Relative expression of Matrin3, RBM20, and ZNF638 across human tissues. Darker red correlates with high expression levels and light red with low expression. Data are taken from The Human Protein Atlas.
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Proposed model for splicing regulation by Matrin3. Cartoon depicting an exon not regulated by Matrin3 (a) and one regulated by Matrin3 (b). The presence of Matrin3‐binding site (b—boxed sequence) on the introns flanking the exon recruits the Matrin3 molecules, which can be bound or not to other splicing regulators like PTBP1.
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RNA in Disease and Development > RNA in Disease
RNA Processing > Splicing Regulation/Alternative Splicing
RNA Interactions with Proteins and Other Molecules > Protein–RNA Interactions: Functional Implications

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