Mitochondrial disorders of the OXPHOS system

E Fernandez‐Vizarra, M Zeviani - FEBS letters, 2021 - Wiley Online Library
Mitochondrial disorders are among the most frequent inborn errors of metabolism, their
primary cause being the dysfunction of the oxidative phosphorylation system (OXPHOS) …

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 …

Defective metabolic programming impairs early neuronal morphogenesis in neural cultures and an organoid model of Leigh syndrome

G Inak, A Rybak-Wolf, P Lisowski, TM Pentimalli… - Nature …, 2021 - nature.com
Leigh syndrome (LS) is a severe manifestation of mitochondrial disease in children and is
currently incurable. The lack of effective models hampers our understanding of the …

[HTML][HTML] Cytochrome c oxidase deficiency

M Brischigliaro, M Zeviani - Biochimica et Biophysica Acta (BBA) …, 2021 - Elsevier
Cytochrome c oxidase (COX) deficiency is characterized by a high degree of genetic and
phenotypic heterogeneity, partly reflecting the extreme structural complexity, multiple post …

[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 …

From synthesis to utilization: The ins and outs of mitochondrial heme

SA Swenson, CM Moore, JR Marcero, AE Medlock… - Cells, 2020 - mdpi.com
Heme is a ubiquitous and essential iron containing metallo-organic cofactor required for
virtually all aerobic life. Heme synthesis is initiated and completed in mitochondria, followed …

Mitochondrial protein synthesis, import, and assembly

TD Fox - Genetics, 2012 - academic.oup.com
The mitochondrion is arguably the most complex organelle in the budding yeast cell
cytoplasm. It is essential for viability as well as respiratory growth. Its innermost aqueous …

[HTML][HTML] Biogenesis and assembly of eukaryotic cytochrome c oxidase catalytic core

IC Soto, F Fontanesi, J Liu, A Barrientos - Biochimica Et Biophysica Acta …, 2012 - Elsevier
Eukaryotic cytochrome c oxidase (COX) is the terminal enzyme of the mitochondrial
respiratory chain. COX is a multimeric enzyme formed by subunits of dual genetic origin …

The Complexity of Mitochondrial Complex IV: An Update of Cytochrome c Oxidase Biogenesis in Plants

N Mansilla, S Racca, DE Gras, DH Gonzalez… - International journal of …, 2018 - mdpi.com
Mitochondrial respiration is an energy producing process that involves the coordinated
action of several protein complexes embedded in the inner membrane to finally produce …

Inventory control: cytochrome c oxidase assembly regulates mitochondrial translation

DU Mick, TD Fox, P Rehling - Nature reviews Molecular cell biology, 2011 - nature.com
Mitochondria maintain genome and translation machinery to synthesize a small subset of
subunits of the oxidative phosphorylation system. To build up functional enzymes, these …