biostudies-literatureNguyen TGNovo Nordisk Fonden (Novo Nordisk Foundation)Novo Nordisk Foundation Center for Protein ResearchNovo Nordisk Fonden7991https://www.ebi.ac.uk/biostudies/studies/S-EPMC10693566biostudies-literatureUnknown14(1)Mitochondria contain their own genetic information and a dedicated translation system to express it. The mitochondrial ribosome is assembled from mitochondrial-encoded RNA and nuclear-encoded ribosomal proteins. Assembly is coordinated in the mitochondrial matrix by biogenesis factors that transiently associate with the maturing particle. Here, we present a structural snapshot of a large mitoribosomal subunit assembly intermediate containing 7 biogenesis factors including the GTPases GTPBP7 and GTPBP10. Our structure illustrates how GTPBP10 aids the folding of the ribosomal RNA during the biogenesis process, how this process is related to bacterial ribosome biogenesis, and why mitochondria require two biogenesis factors in contrast to only one in bacteria.Nature communicationsStructural insights into the role of GTPBP10 in the RNA maturation of the mitoribosome.PMC10693566PI Eva KummerNNF21OC0067360Ritter CNguyen TGKummer EfalseStructural insights into the role of GTPBP10 in the RNA maturation of the mitoribosome.Mitochondria contain their own genetic information and a dedicated translation system to express it. The mitochondrial ribosome is assembled from mitochondrial-encoded RNA and nuclear-encoded ribosomal proteins. Assembly is coordinated in the mitochondrial matrix by biogenesis factors that transiently associate with the maturing particle. Here, we present a structural snapshot of a large mitoribosomal subunit assembly intermediate containing 7 biogenesis factors including the GTPases GTPBP7 and GTPBP10. Our structure illustrates how GTPBP10 aids the folding of the ribosomal RNA during the biogenesis process, how this process is related to bacterial ribosome biogenesis, and why mitochondria require two biogenesis factors in contrast to only one in bacteria.2023-01-01T00:00:00Z2023 Dec2026-05-29T07:37:49.439Z2025-05-29T21:46:32.001ZS-EPMC106935663804294910.1038/s41467-023-43599-z