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 …

[HTML][HTML] 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 …

Bicyclobutane carboxylic amide as a cysteine-directed strained electrophile for selective targeting of proteins

K Tokunaga, M Sato, K Kuwata, C Miura… - Journal of the …, 2020 - ACS Publications
Expanding the repertoire of electrophiles with unique reactivity features would facilitate the
development of covalent inhibitors with desirable reactivity profiles. We herein introduce …

Mitochondrial ribosome assembly in health and disease

D De Silva, YT Tu, A Amunts, F Fontanesi… - Cell Cycle, 2015 - Taylor & Francis
The ribosome is a structurally and functionally conserved macromolecular machine
universally responsible for catalyzing protein synthesis. Within eukaryotic cells, mitochondria …

Mitochondrial ribosomes in cancer

HJ Kim, P Maiti, A Barrientos - Seminars in cancer biology, 2017 - Elsevier
Mitochondria play fundamental roles in the regulation of life and death of eukaryotic cells.
They mediate aerobic energy conversion through the oxidative phosphorylation (OXPHOS) …

ATAD3 gene cluster deletions cause cerebellar dysfunction associated with altered mitochondrial DNA and cholesterol metabolism

R Desai, AE Frazier, R Durigon, H Patel, AW Jones… - Brain, 2017 - academic.oup.com
Although mitochondrial disorders are clinically heterogeneous, they frequently involve the
central nervous system and are among the most common neurogenetic disorders …

[HTML][HTML] The process of mammalian mitochondrial protein synthesis

N Mai, ZMA Chrzanowska-Lightowlers… - Cell and tissue …, 2017 - Springer
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 …

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 …

[HTML][HTML] The human mitochondrial DEAD-box protein DDX28 resides in RNA granules and functions in mitoribosome assembly

YT Tu, A Barrientos - Cell reports, 2015 - cell.com
Human mitochondrial ribosomes are specialized in the synthesis of 13 proteins, which are
fundamental components of the oxidative phosphorylation system. The pathway of …

Yeast as a system for modeling mitochondrial disease mechanisms and discovering therapies

JP Lasserre, A Dautant, RS Aiyar… - Disease models & …, 2015 - journals.biologists.com
Mitochondrial diseases are severe and largely untreatable. Owing to the many essential
processes carried out by mitochondria and the complex cellular systems that support these …