{"database":"biostudies-literature","file_versions":[],"scores":null,"additional":{"submitter":["Kim S"],"funding":["NIDCR NIH HHS"],"pagination":["2935-2948.e7"],"full_dataset_link":["https://www.ebi.ac.uk/biostudies/studies/S-EPMC11316625"],"repository":["biostudies-literature"],"omics_type":["Unknown"],"volume":["84(15)"],"pubmed_abstract":["Mitochondria are essential regulators of innate immunity. They generate long mitochondrial double-stranded RNAs (mt-dsRNAs) and release them into the cytosol to trigger an immune response under pathological stress conditions. Yet the regulation of these self-immunogenic RNAs remains largely unknown. Here, we employ CRISPR screening on mitochondrial RNA (mtRNA)-binding proteins and identify NOP2/Sun RNA methyltransferase 4 (NSUN4) as a key regulator of mt-dsRNA expression in human cells. We find that NSUN4 induces 5-methylcytosine (m<sup>5</sup>C) modification on mtRNAs, especially on the termini of light-strand long noncoding RNAs. These m<sup>5</sup>C-modified RNAs are recognized by complement C1q-binding protein (C1QBP), which recruits polyribonucleotide nucleotidyltransferase to facilit"],"journal":["Molecular cell"],"pubmed_title":["RNA 5-methylcytosine marks mitochondrial double-stranded RNAs for degradation and cytosolic release."],"pmcid":["PMC11316625"],"funding_grant_id":["R01 DE032707"],"pubmed_authors":["Kim S","You K","Widowati TA","Ku J","Tan S","Lee K","Kim Y","Ku D"],"additional_accession":[]},"is_claimable":false,"name":"RNA 5-methylcytosine marks mitochondrial double-stranded RNAs for degradation and cytosolic release.","description":"Mitochondria are essential regulators of innate immunity. They generate long mitochondrial double-stranded RNAs (mt-dsRNAs) and release them into the cytosol to trigger an immune response under pathological stress conditions. Yet the regulation of these self-immunogenic RNAs remains largely unknown. Here, we employ CRISPR screening on mitochondrial RNA (mtRNA)-binding proteins and identify NOP2/Sun RNA methyltransferase 4 (NSUN4) as a key regulator of mt-dsRNA expression in human cells. We find that NSUN4 induces 5-methylcytosine (m<sup>5</sup>C) modification on mtRNAs, especially on the termini of light-strand long noncoding RNAs. These m<sup>5</sup>C-modified RNAs are recognized by complement C1q-binding protein (C1QBP), which recruits polyribonucleotide nucleotidyltransferase to facilit","dates":{"release":"2024-01-01T00:00:00Z","publication":"2024 Aug","modification":"2026-04-12T19:30:18.668Z","creation":"2026-04-07T13:21:05.838Z"},"accession":"S-EPMC11316625","cross_references":{"pubmed":["39019044"],"doi":["10.1016/j.molcel.2024.06.023"]}}