title: The process of mammalian mitochondrial protein synthesis.
abstract: Oxidative phosphorylation (OXPHOS) is the mechanism whereby ATP, the major energy source for the cell, is produced by harnessing cellular respiration in the mitochondrion. This is facilitated by five multi-subunit complexes housed within the inner mitochondrial membrane. These complexes, with the exception of complex II, are of a dual genetic origin, requiring expression from nuclear and mitochondrial genes. Mitochondrially encoded mRNA is translated on the mitochondrial ribosome (mitoribosome) and the recent release of the near atomic resolution structure of the mammalian mitoribosome has highlighted its peculiar features. However, whereas some aspects of mitochondrial translation are understood, much is to be learnt about the presentation of mitochondrial mRNA to the mitoribosome, the biogenesis of the machinery, the exact role of the membrane, the constitution of the translocon/insertion machinery and the regulation of translation in the mitochondrion. This review addresses our current knowledge of mammalian mitochondrial gene expression, highlights key questions and indicates how defects in this process can result in profound mitochondrial disease.
authors.affiliation: Wellcome Trust Centre for Mitochondrial Research, Institute of Neuroscience, Medical School, Newcastle University, Newcastle upon Tyne, NE2 4HH, UK.
authors.forename: Nicole
authors.initials: N
authors.lastname: Mai
authors.affiliation: Wellcome Trust Centre for Mitochondrial Research, Institute of Neuroscience, Medical School, Newcastle University, Newcastle upon Tyne, NE2 4HH, UK. zofia.chrzanowska-lightowlers@ncl.ac.uk.|Wellcome Trust Centre for Mitochondrial Research, Institute for Cell and Molecular Biosciences, Medical School, Newcastle University, Newcastle upon Tyne, NE2 4HH, UK. zofia.chrzanowska-lightowlers@ncl.ac.uk.
authors.forename: Zofia M A
authors.initials: ZM
authors.lastname: Chrzanowska-Lightowlers
authors.affiliation: Wellcome Trust Centre for Mitochondrial Research, Institute for Cell and Molecular Biosciences, Medical School, Newcastle University, Newcastle upon Tyne, NE2 4HH, UK. robert.lightowlers@ncl.ac.uk.
authors.forename: Robert N
authors.initials: RN
authors.lastname: Lightowlers
mesh_terms: D000818:Animals; D006801:Humans; D008322:Mammals / Q000378:metabolism*; D024101:Mitochondrial Proteins / Q000096:biosynthesis*; D000069396:Mitochondrial Ribosomes / Q000378:metabolism; D008954:Models, Biological; D014176:Protein Biosynthesis
chemical_list: D024101:Mitochondrial Proteins
keywords: Mitochondria; Mitochondrial diseases; Mitochondrial translation; Mitoribosome; Protein synthesis