Mitochondrial protein synthesis: figuring the fundamentals, complexities and complications, of mammalian mitochondrial translation

FEBS Lett. 2014 Aug 1;588(15):2496-503. doi: 10.1016/j.febslet.2014.05.054. Epub 2014 Jun 6.


Mitochondrial protein synthesis is essential for all mammals, being responsible for providing key components of the oxidative phosphorylation complexes. Although only thirteen different polypeptides are made, the molecular details of this deceptively simple process remain incomplete. Central to this process is a non-canonical ribosome, the mitoribosome, which has evolved to address its unique mandate. In this review, we integrate the current understanding of the molecular aspects of mitochondrial translation with recent advances in structural biology. We identify numerous key questions that we will need to answer if we are to increase our knowledge of the molecular mechanisms underlying mitochondrial protein synthesis.

Keywords: Gene expression; Mitochondria; RNA; Ribosomes; Translation.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Animals
  • Humans
  • Mitochondria / metabolism*
  • Mitochondrial Proteins / genetics
  • Mitochondrial Proteins / metabolism*
  • Protein Biosynthesis*
  • RNA / genetics
  • RNA / metabolism
  • RNA, Mitochondrial
  • Ribosomal Proteins / genetics
  • Ribosomal Proteins / metabolism*
  • Ribosomes / metabolism


  • Mitochondrial Proteins
  • RNA, Mitochondrial
  • Ribosomal Proteins
  • RNA