Structural basis for late maturation steps of the human mitoribosomal large subunit

Nat Commun. 2021 Jun 16;12(1):3673. doi: 10.1038/s41467-021-23617-8.

Abstract

Mitochondrial ribosomes (mitoribosomes) synthesize a critical set of proteins essential for oxidative phosphorylation. Therefore, mitoribosomal function is vital to the cellular energy supply. Mitoribosome biogenesis follows distinct molecular pathways that remain poorly understood. Here, we determine the cryo-EM structures of mitoribosomes isolated from human cell lines with either depleted or overexpressed mitoribosome assembly factor GTPBP5, allowing us to capture consecutive steps during mitoribosomal large subunit (mt-LSU) biogenesis. Our structures provide essential insights into the last steps of 16S rRNA folding, methylation and peptidyl transferase centre (PTC) completion, which require the coordinated action of nine assembly factors. We show that mammalian-specific MTERF4 contributes to the folding of 16S rRNA, allowing 16 S rRNA methylation by MRM2, while GTPBP5 and NSUN4 promote fine-tuning rRNA rearrangements leading to PTC formation. Moreover, our data reveal an unexpected involvement of the elongation factor mtEF-Tu in mt-LSU assembly, where mtEF-Tu interacts with GTPBP5, similar to its interaction with tRNA during translational elongation.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Cell Line
  • Cryoelectron Microscopy
  • Humans
  • Methyltransferases / chemistry
  • Methyltransferases / metabolism
  • Mitochondrial Ribosomes / chemistry*
  • Mitochondrial Ribosomes / metabolism
  • Models, Molecular
  • Monomeric GTP-Binding Proteins / chemistry
  • Monomeric GTP-Binding Proteins / metabolism
  • Multiprotein Complexes
  • Peptide Elongation Factor Tu / chemistry
  • Peptide Elongation Factor Tu / metabolism
  • Peptidyl Transferases / chemistry
  • Peptidyl Transferases / metabolism
  • Protein Binding
  • RNA Folding
  • RNA, Ribosomal, 16S / chemistry
  • RNA, Ribosomal, 16S / metabolism
  • Ribosome Subunits, Large / chemistry*
  • Ribosome Subunits, Large / metabolism
  • Transcription Factors / chemistry
  • Transcription Factors / metabolism

Substances

  • MTERF4 protein, human
  • Multiprotein Complexes
  • RNA, Ribosomal, 16S
  • Transcription Factors
  • MRM2 protein, human
  • Methyltransferases
  • NSUN4 protein, human
  • Peptidyl Transferases
  • Peptide Elongation Factor Tu
  • MTG2 protein, human
  • Monomeric GTP-Binding Proteins