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2002 2
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A late-stage assembly checkpoint of the human mitochondrial ribosome large subunit.
Rebelo-Guiomar P, Pellegrino S, Dent KC, Sas-Chen A, Miller-Fleming L, Garone C, Van Haute L, Rogan JF, Dinan A, Firth AE, Andrews B, Whitworth AJ, Schwartz S, Warren AJ, Minczuk M. Rebelo-Guiomar P, et al. Nat Commun. 2022 Feb 17;13(1):929. doi: 10.1038/s41467-022-28503-5. Nat Commun. 2022. PMID: 35177605 Free PMC article.
Here, a genome-wide analysis of the human mitochondrial transcriptome shows that 2'-O-methylation is limited to residues of the mitoribosomal large subunit (mtLSU) 16S mt-rRNA, introduced by MRM1, MRM2 and MRM3, with the modifications installed by the latter two pro …
Here, a genome-wide analysis of the human mitochondrial transcriptome shows that 2'-O-methylation is limited to residues of the mitoribosoma …
MRM2 and MRM3 are involved in biogenesis of the large subunit of the mitochondrial ribosome.
Rorbach J, Boesch P, Gammage PA, Nicholls TJ, Pearce SF, Patel D, Hauser A, Perocchi F, Minczuk M. Rorbach J, et al. Mol Biol Cell. 2014 Sep 1;25(17):2542-55. doi: 10.1091/mbc.E14-01-0014. Epub 2014 Jul 9. Mol Biol Cell. 2014. PMID: 25009282 Free PMC article.
Here we investigate two methyltransferases, MRM2 (also known as RRMJ2, encoded by FTSJ2) and MRM3 (also known as RMTL1, encoded by RNMTL1), that are responsible for modification of nucleotides of the 16S rRNA A-loop, an essential component of the peptidyl transferas …
Here we investigate two methyltransferases, MRM2 (also known as RRMJ2, encoded by FTSJ2) and MRM3 (also known as RMTL1, encoded by RN …
Defective mitochondrial rRNA methyltransferase MRM2 causes MELAS-like clinical syndrome.
Garone C, D'Souza AR, Dallabona C, Lodi T, Rebelo-Guiomar P, Rorbach J, Donati MA, Procopio E, Montomoli M, Guerrini R, Zeviani M, Calvo SE, Mootha VK, DiMauro S, Ferrero I, Minczuk M. Garone C, et al. Hum Mol Genet. 2017 Nov 1;26(21):4257-4266. doi: 10.1093/hmg/ddx314. Hum Mol Genet. 2017. PMID: 28973171 Free PMC article.
Targeted exome sequencing of genes encoding the mitochondrial proteome identified a damaging mutation, c.567 G > A, affecting a highly conserved amino acid residue (p.Gly189Arg) of the MRM2 protein. MRM2 has never before been linked to a human disease and encodes …
Targeted exome sequencing of genes encoding the mitochondrial proteome identified a damaging mutation, c.567 G > A, affecting a highly co …
The yeast GTPase Mtg2p is required for mitochondrial translation and partially suppresses an rRNA methyltransferase mutant, mrm2.
Datta K, Fuentes JL, Maddock JR. Datta K, et al. Mol Biol Cell. 2005 Feb;16(2):954-63. doi: 10.1091/mbc.e04-07-0622. Epub 2004 Dec 9. Mol Biol Cell. 2005. PMID: 15591131 Free PMC article.
Significantly, elevated levels of Mtg2p partially suppress the thermosensitive loss of mitochondrial DNA in a 21S rRNA methyltransferase mutant, mrm2. We propose that Mtg2p is involved in mitochondrial ribosome biogenesis. ...
Significantly, elevated levels of Mtg2p partially suppress the thermosensitive loss of mitochondrial DNA in a 21S rRNA methyltransfer …
MRM2 encodes a novel yeast mitochondrial 21S rRNA methyltransferase.
Pintard L, Bujnicki JM, Lapeyre B, Bonnerot C. Pintard L, et al. EMBO J. 2002 Mar 1;21(5):1139-47. doi: 10.1093/emboj/21.5.1139. EMBO J. 2002. PMID: 11867542 Free PMC article.
We demonstrate here that the yeast nuclear genome encodes a mitochondrial protein, named Mrm2, which is required for methylating U(2791) of 21S rRNA, both in vivo and in vitro. Deletion of the MRM2 gene causes thermosensitive respiration and leads to rapid lo …
We demonstrate here that the yeast nuclear genome encodes a mitochondrial protein, named Mrm2, which is required for methylating U(27 …
Human GTPBP5 (MTG2) fuels mitoribosome large subunit maturation by facilitating 16S rRNA methylation.
Maiti P, Antonicka H, Gingras AC, Shoubridge EA, Barrientos A. Maiti P, et al. Nucleic Acids Res. 2020 Aug 20;48(14):7924-7943. doi: 10.1093/nar/gkaa592. Nucleic Acids Res. 2020. PMID: 32652011 Free PMC article.
We show that a fraction of GTPBP5 co-sediments with the large mitoribosome subunit (mtLSU), and crosslinks specifically with the 16S rRNA, and several mtLSU proteins and assembly factors. Notably, the latter group includes MTERF4, involved in monosome assembly, and MRM2
We show that a fraction of GTPBP5 co-sediments with the large mitoribosome subunit (mtLSU), and crosslinks specifically with the 16S rRNA
Stepwise maturation of the peptidyl transferase region of human mitoribosomes.
Lenarčič T, Jaskolowski M, Leibundgut M, Scaiola A, Schönhut T, Saurer M, Lee RG, Rackham O, Filipovska A, Ban N. Lenarčič T, et al. Nat Commun. 2021 Jun 16;12(1):3671. doi: 10.1038/s41467-021-23811-8. Nat Commun. 2021. PMID: 34135320 Free PMC article.
We discover that the NSUN4-MTERF4 dimer plays a critical role in the process by stabilizing the 16S rRNA in a conformation that exposes the functionally important regions of rRNA for modification by the MRM2 methyltransferase and quality control interactions …
We discover that the NSUN4-MTERF4 dimer plays a critical role in the process by stabilizing the 16S rRNA in a conformation that expos …
Structural basis for late maturation steps of the human mitoribosomal large subunit.
Cipullo M, Gesé GV, Khawaja A, Hällberg BM, Rorbach J. Cipullo M, et al. Nat Commun. 2021 Jun 16;12(1):3673. doi: 10.1038/s41467-021-23617-8. Nat Commun. 2021. PMID: 34135318 Free PMC article.
Our structures provide essential insights into the last steps of 16S rRNA folding, methylation and peptidyl transferase centre (PTC) completion, which require the coordinated action of nine assembly factors. We show that mammalian-specific MTERF4 contributes to the folding …
Our structures provide essential insights into the last steps of 16S rRNA folding, methylation and peptidyl transferase centre (PTC) …
Functional redundancy of Spb1p and a snR52-dependent mechanism for the 2'-O-ribose methylation of a conserved rRNA position in yeast.
Bonnerot C, Pintard L, Lutfalla G. Bonnerot C, et al. Mol Cell. 2003 Nov;12(5):1309-15. doi: 10.1016/s1097-2765(03)00435-0. Mol Cell. 2003. PMID: 14636587 Free article.
In yeast, guide snoRNAs have been assigned to 51 of the 55 rRNA ribose methylation sites. LSU-Um2918 is one of the four remaining positions. This residue is highly conserved and located in the peptidyl transferase center of the ribosome. The equivalent position on the E. c …
In yeast, guide snoRNAs have been assigned to 51 of the 55 rRNA ribose methylation sites. LSU-Um2918 is one of the four remaining pos …
Assignment of 2'-O-methyltransferases to modification sites on the mammalian mitochondrial large subunit 16 S ribosomal RNA (rRNA).
Lee KW, Bogenhagen DF. Lee KW, et al. J Biol Chem. 2014 Sep 5;289(36):24936-42. doi: 10.1074/jbc.C114.581868. Epub 2014 Jul 29. J Biol Chem. 2014. PMID: 25074936 Free PMC article.
Advances in proteomics and large scale studies of potential mitochondrial proteins have led to the identification of many novel mitochondrial proteins in need of further characterization. Among these novel proteins are three mammalian rRNA methyltransferase family members …
Advances in proteomics and large scale studies of potential mitochondrial proteins have led to the identification of many novel mitochondria …
13 results