Mechanisms and regulation of protein synthesis in mitochondria

E Kummer, N Ban - Nature Reviews Molecular Cell Biology, 2021 - nature.com
Mitochondria are cellular organelles responsible for generation of chemical energy in the
process called oxidative phosphorylation. They originate from a bacterial ancestor and …

Organization and expression of the mammalian mitochondrial genome

O Rackham, A Filipovska - Nature Reviews Genetics, 2022 - nature.com
The mitochondrial genome encodes core subunits of the respiratory chain that drives
oxidative phosphorylation and is, therefore, essential for energy conversion. Advances in …

Mitochondrial diseases: the contribution of organelle stress responses to pathology

A Suomalainen, BJ Battersby - Nature reviews Molecular cell biology, 2018 - nature.com
Mitochondrial diseases affect one in 2,000 individuals; they can present at any age and they
can manifest in any organ. How defects in mitochondria can cause such a diverse range of …

Mitochondrial transcription and translation: overview

AR D'Souza, M Minczuk - Essays in biochemistry, 2018 - portlandpress.com
Mitochondria are the major source of ATP in the cell. Five multi-subunit complexes in the
inner membrane of the organelle are involved in the oxidative phosphorylation required for …

Mitochondrial genome engineering: the revolution may not be CRISPR-Ized

PA Gammage, CT Moraes, M Minczuk - Trends in Genetics, 2018 - cell.com
In recent years mitochondrial DNA (mtDNA) has transitioned to greater prominence across
diverse areas of biology and medicine. The recognition of mitochondria as a major …

Mitochondrial energy generation disorders: genes, mechanisms, and clues to pathology

AE Frazier, DR Thorburn, AG Compton - Journal of Biological Chemistry, 2019 - ASBMB
Inherited disorders of oxidative phosphorylation cause the clinically and genetically
heterogeneous diseases known as mitochondrial energy generation disorders, or …

Regulation of mammalian mitochondrial gene expression: recent advances

SF Pearce, P Rebelo-Guiomar, AR D'Souza… - Trends in biochemical …, 2017 - cell.com
Perturbation of mitochondrial DNA (mtDNA) gene expression can lead to human
pathologies. Therefore, a greater appreciation of the basic mechanisms of mitochondrial …

METTL15 introduces N4-methylcytidine into human mitochondrial 12S rRNA and is required for mitoribosome biogenesis

LV Haute, AG Hendrick, AR D'Souza… - Nucleic acids …, 2019 - academic.oup.com
Post-transcriptional RNA modifications, the epitranscriptome, play important roles in
modulating the functions of RNA species. Modifications of rRNA are key for ribosome …

The diseased mitoribosome

A Ferrari, S Del'Olio, A Barrientos - FEBS letters, 2021 - Wiley Online Library
Mitochondria control life and death in eukaryotic cells. Harboring a unique circular genome,
a by‐product of an ancient endosymbiotic event, mitochondria maintains a specialized and …

Biallelic mutations in MRPS34 lead to instability of the small mitoribosomal subunit and Leigh syndrome

NJ Lake, BD Webb, DA Stroud, TR Richman… - The American Journal of …, 2017 - cell.com
The synthesis of all 13 mitochondrial DNA (mtDNA)-encoded protein subunits of the human
oxidative phosphorylation (OXPHOS) system is carried out by mitochondrial ribosomes …