{"database":"biostudies-literature","file_versions":[],"scores":null,"additional":{"submitter":["Foged MM"],"funding":["HHMI","HHMI (HHMI)"],"pagination":["e2414187121"],"full_dataset_link":["https://www.ebi.ac.uk/biostudies/studies/S-EPMC11588129"],"repository":["biostudies-literature"],"omics_type":["Unknown"],"volume":["121(47)"],"pubmed_abstract":["Mitochondrial biogenesis relies on both the nuclear and mitochondrial genomes, and imbalance in their expression can lead to inborn errors of metabolism, inflammation, and aging. Here, we investigate N6AMT1, a nucleo-cytosolic methyltransferase that exhibits genetic codependency with mitochondria. We determine transcriptional and translational profiles of <i>N6AMT1</i> and report that it is required for the cytosolic translation of TRMT10C (MRPP1) and PRORP (MRPP3), two subunits of the mitochondrial RNAse P enzyme. In the absence of <i>N6AMT1</i>, or when its catalytic activity is abolished, RNA processing within mitochondria is impaired, leading to the accumulation of unprocessed and double-stranded RNA, thus preventing mitochondrial protein synthesis and oxidative phosphorylation, and le"],"journal":["Proceedings of the National Academy of Sciences of the United States of America"],"pubmed_title":["Cytosolic <i>N6AMT1-</i>dependent translation supports mitochondrial RNA processing."],"pmcid":["PMC11588129"],"funding_grant_id":["DRG-2250-16"],"pubmed_authors":["Lisci M","Jourdain AA","Foged MM","Mootha VK","Boutguetait D","Zinshteyn B","Munch C","Allen GE","Recazens E","Chollet S"],"additional_accession":[]},"is_claimable":false,"name":"Cytosolic <i>N6AMT1-</i>dependent translation supports mitochondrial RNA processing.","description":"Mitochondrial biogenesis relies on both the nuclear and mitochondrial genomes, and imbalance in their expression can lead to inborn errors of metabolism, inflammation, and aging. Here, we investigate N6AMT1, a nucleo-cytosolic methyltransferase that exhibits genetic codependency with mitochondria. We determine transcriptional and translational profiles of <i>N6AMT1</i> and report that it is required for the cytosolic translation of TRMT10C (MRPP1) and PRORP (MRPP3), two subunits of the mitochondrial RNAse P enzyme. In the absence of <i>N6AMT1</i>, or when its catalytic activity is abolished, RNA processing within mitochondria is impaired, leading to the accumulation of unprocessed and double-stranded RNA, thus preventing mitochondrial protein synthesis and oxidative phosphorylation, and le","dates":{"release":"2024-01-01T00:00:00Z","publication":"2024 Nov","modification":"2026-06-02T06:23:22.803Z","creation":"2025-04-04T09:58:36.868Z"},"accession":"S-EPMC11588129","cross_references":{"pubmed":["39503847"],"doi":["10.1073/pnas.2414187121"]}}