{"type": "root", "children": [{"type": "p", "children": [{"type": "t", "text": "\nMRM3, also known as RMTL1 and encoded by RNMTL1, is a mitochondrial RNA methyltransferase essential for the biogenesis and function of the large subunit of the mitoribosome. It catalyzes the methylation of nucleotides within the A‐loop of the 16S rRNA—a key structural element of the peptidyl transferase center—and thus facilitates proper ribosomal assembly and efficient mitochondrial protein synthesis. Deficiency in MRM3 leads to aberrant assembly of the mitoribosome, impaired mitochondrial translation, and ultimately respiratory incompetence in human cells."}, {"type": "fg", "children": [{"type": "fg_f", "ref": "1"}]}, {"type": "t", "text": "MRM3 has been further characterized through proteomic and site‐specific RNA modification analyses. In these studies, MRM3 (along with its related family members) was shown to localize near mitochondrial nucleoids and to specifically modify the large subunit rRNA at residue G(1370). This precise nucleotide modification underscores the conserved and critical function of MRM3 in ensuring the structural integrity of the mitoribosome."}, {"type": "fg", "children": [{"type": "fg_f", "ref": "2"}]}, {"type": "t", "text": "In addition to its enzymatic role in rRNA maturation, the transcriptional regulation of the RNMTL1 gene has been dissected to reveal that its strong expression relies predominantly on an ATF/CREB binding element within its promoter. The secondary structure of this element appears vital for the interaction with its cognate transcription factors, and disruptions in this regulatory mechanism could impact mitochondrial function and contribute to oncogenic processes."}, {"type": "fg", "children": [{"type": "fg_f", "ref": "3"}]}, {"type": "t", "text": ""}]}, {"type": "rg", "children": [{"type": "r", "ref": 1, "children": [{"type": "t", "text": "Joanna Rorbach, Pierre Boesch, Payam A Gammage, et al. "}, {"type": "b", "children": [{"type": "t", "text": "MRM2 and MRM3 are involved in biogenesis of the large subunit of the mitochondrial ribosome."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Mol Biol Cell (2014)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1091/mbc.E14-01-0014"}], "href": "https://doi.org/10.1091/mbc.E14-01-0014"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "25009282"}], "href": "https://pubmed.ncbi.nlm.nih.gov/25009282"}]}, {"type": "r", "ref": 2, "children": [{"type": "t", "text": "Ken-Wing Lee, Daniel F Bogenhagen "}, {"type": "b", "children": [{"type": "t", "text": "Assignment of 2'-O-methyltransferases to modification sites on the mammalian mitochondrial large subunit 16 S ribosomal RNA (rRNA)."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "J Biol Chem (2014)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1074/jbc.C114.581868"}], "href": "https://doi.org/10.1074/jbc.C114.581868"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "25074936"}], "href": "https://pubmed.ncbi.nlm.nih.gov/25074936"}]}, {"type": "r", "ref": 3, "children": [{"type": "t", "text": "Jian Xu, Jing De Zhu, Min Ni, et al. "}, {"type": "b", "children": [{"type": "t", "text": "The ATF/CREB site is the key element for transcription of the human RNA methyltransferase like 1(RNMTL1) gene, a newly discovered 17p13.3 gene."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Cell Res (2002)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1038/sj.cr.7290124"}], "href": "https://doi.org/10.1038/sj.cr.7290124"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "12296377"}], "href": "https://pubmed.ncbi.nlm.nih.gov/12296377"}]}]}]}