Structural 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. The biogenesis of ribosomes is a highly coordinated process. Here, Nguyen et al. uncover how the mitochondria-specific interplay of the GTPases GTPBP10 and GTPBP7 ensures proper maturation of the catalytic RNA center of the human mitoribosome.

Automated summary

Mitochondria contain their own genetic information and translation system. Researchers have discovered that GTPBP10 plays a role in the folding of mitochondrial ribosomal RNA during the biogenesis process, which is related to bacterial ribosome biogenesis. Unlike bacteria, mitochondria require two biogenesis factors. This study reveals the process of maturation in the human mitoribosome and the interplay between GTPBP10 and GTPBP7.