The assembly, regulation and function of the mitochondrial respiratory chain

I Vercellino, LA Sazanov - Nature reviews Molecular cell biology, 2022 - nature.com
The mitochondrial oxidative phosphorylation system is central to cellular metabolism. It
comprises five enzymatic complexes and two mobile electron carriers that work in a …

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] Getting out what you put in: Copper in mitochondria and its impacts on human disease

PA Cobine, SA Moore, SC Leary - … Et Biophysica Acta (BBA)-Molecular Cell …, 2021 - Elsevier
Mitochondria accumulate copper in their matrix for the eventual maturation of the
cuproenzymes cytochrome c oxidase and superoxide dismutase. Transport into the matrix is …

Assembly of mammalian oxidative phosphorylation complexes I–V and supercomplexes

A Signes, E Fernandez-Vizarra - Essays in biochemistry, 2018 - portlandpress.com
The assembly of the five oxidative phosphorylation system (OXPHOS) complexes in the
inner mitochondrial membrane is an intricate process. The human enzymes comprise core …

Mitochondrial cytochrome c oxidase biogenesis: Recent developments

A Timón-Gómez, E Nývltová, LA Abriata, AJ Vila… - Seminars in cell & …, 2018 - Elsevier
Mitochondrial cytochrome c oxidase (COX) is the primary site of cellular oxygen
consumption and is essential for aerobic energy generation in the form of ATP. Human COX …

Organization and regulation of mitochondrial protein synthesis

M Ott, A Amunts, A Brown - Annual review of biochemistry, 2016 - annualreviews.org
Mitochondria are essential organelles of endosymbiotic origin that are responsible for
oxidative phosphorylation within eukaryotic cells. Independent evolution between species …

Mitochondrial OXPHOS biogenesis: co-regulation of protein synthesis, import, and assembly pathways

JX Tang, K Thompson, RW Taylor… - International Journal of …, 2020 - mdpi.com
The assembly of mitochondrial oxidative phosphorylation (OXPHOS) complexes is an
intricate process, which—given their dual-genetic control—requires tight co-regulation of two …

SDH5, a Gene Required for Flavination of Succinate Dehydrogenase, Is Mutated in Paraganglioma

HX Hao, O Khalimonchuk, M Schraders, N Dephoure… - Science, 2009 - science.org
Mammalian mitochondria contain about 1100 proteins, nearly 300 of which are
uncharacterized. Given the well-established role of mitochondrial defects in human disease …

[HTML][HTML] Structure, function, and assembly of heme centers in mitochondrial respiratory complexes

HJ Kim, O Khalimonchuk, PM Smith… - Biochimica et Biophysica …, 2012 - Elsevier
The sequential flow of electrons in the respiratory chain, from a low reduction potential
substrate to O2, is mediated by protein-bound redox cofactors. In mitochondria, hemes …

MITRAC links mitochondrial protein translocation to respiratory-chain assembly and translational regulation

DU Mick, S Dennerlein, H Wiese, R Reinhold… - Cell, 2012 - cell.com
Mitochondrial respiratory-chain complexes assemble from subunits of dual genetic origin
assisted by specialized assembly factors. Whereas core subunits are translated on …