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Mitochondrial RNA polymerase produces long polycistronic precursors that contain the mRNAs, rRNAs and tRNAs needed for mitochondrial translation. Mitochondrial RNase P (mt-RNase P) initiates the maturation of the precursors by cleaving at the 5′ ends of the tRNAs. Human mt-RNase P is only active as a tripartite complex (mitochondrial RNase P proteins 1–3; MRPP1-3), whereas plant and trypanosomal RNase Ps (PRORPs)—albeit homologous to MRPP3—are active as single proteins. The reason for this discrepancy has so far remained obscure. Here, we present the crystal structure of human MRPP3, which features a remarkably distorted and hence non-productive active site that we propose will switch to a fully productive state only upon association with MRPP1, MRPP2 and pre-tRNA substrate. We suggest a mechanism in which MRPP1 and MRPP2 both deliver the pre-tRNA substrate and activate MRPP3 through an induced-fit process. 相似文献
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Tobias?B. Haack Robert Kopajtich Peter Freisinger Thomas Wieland Joanna Rorbach Thomas?J. Nicholls Enrico Baruffini Anett Walther Katharina Danhauser Franz?A. Zimmermann Ralf?A. Husain Jessica Schum Helen Mundy Ileana Ferrero Tim?M. Strom Thomas Meitinger Robert?W. Taylor Michal Minczuk Johannes?A. Mayr Holger Prokisch 《American journal of human genetics》2013,93(2):211-223
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Concerted regulation of mitochondrial and nuclear non‐coding RNAs by a dual‐targeted RNase Z
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Stefan J Siira Giulia Rossetti Tara R Richman Kara Perks Judith A Ermer Irina Kuznetsova Laetitia Hughes Anne‐Marie J Shearwood Helena M Viola Livia C Hool Oliver Rackham Aleksandra Filipovska 《EMBO reports》2018,19(10)
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Rossmanith W 《Biochimica et biophysica acta》2012,1819(9-10):1017-1026
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Metodi?D. Metodiev Kyle Thompson Charlotte?L. Alston Andrew?A.M. Morris Langping He Zarah Assouline Marlène Rio Nadia Bahi-Buisson Angela Pyle Helen Griffin Stefan Siira Aleksandra Filipovska Arnold Munnich Patrick?F. Chinnery Robert McFarland Agnès R?tig Robert?W. Taylor 《American journal of human genetics》2016,98(5):993-1000
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