The oxidative phosphorylation system in mammalian mitochondria
S Papa, PL Martino, G Capitanio, A Gaballo… - Advances in …, 2012 - Springer
The chapter provides a review of the state of art of the oxidative phosphorylation system in
mammalian mitochondria. The sections of the paper deal with:(i) the respiratory chain as a …
mammalian mitochondria. The sections of the paper deal with:(i) the respiratory chain as a …
Mitochondrial genetics
PF Chinnery, G Hudson - British medical bulletin, 2013 - academic.oup.com
Introduction In the last 10 years the field of mitochondrial genetics has widened, shifting the
focus from rare sporadic, metabolic disease to the effects of mitochondrial DNA (mtDNA) …
focus from rare sporadic, metabolic disease to the effects of mitochondrial DNA (mtDNA) …
Penalized Cox regression analysis in the high-dimensional and low-sample size settings, with applications to microarray gene expression data
Motivation: An important application of microarray technology is to relate gene expression
profiles to various clinical phenotypes of patients. Success has been demonstrated in …
profiles to various clinical phenotypes of patients. Success has been demonstrated in …
Opa1 overexpression ameliorates the phenotype of two mitochondrial disease mouse models
G Civiletto, T Varanita, R Cerutti, T Gorletta, S Barbaro… - Cell metabolism, 2015 - cell.com
Increased levels of the mitochondria-shaping protein Opa1 improve respiratory chain
efficiency and protect from tissue damage, suggesting that it could be an attractive target to …
efficiency and protect from tissue damage, suggesting that it could be an attractive target to …
[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 …
substrate to O2, is mediated by protein-bound redox cofactors. In mitochondria, hemes …
Fatal infantile cardioencephalomyopathy with COX deficiency and mutations in SCO2, a COX assembly gene
LC Papadopoulou, CM Sue, MM Davidson, K Tanji… - Nature …, 1999 - nature.com
Mammalian cytochrome c oxidase (COX) catalyses the transfer of reducing equivalents from
cytochrome c to molecular oxygen and pumps protons across the inner mitochondrial …
cytochrome c to molecular oxygen and pumps protons across the inner mitochondrial …
MPV17 encodes an inner mitochondrial membrane protein and is mutated in infantile hepatic mitochondrial DNA depletion
A Spinazzola, C Viscomi, E Fernandez-Vizarra… - Nature …, 2006 - nature.com
The mitochondrial (mt) DNA depletion syndromes (MDDS) are genetic disorders
characterized by a severe, tissue-specific decrease of mtDNA copy number, leading to …
characterized by a severe, tissue-specific decrease of mtDNA copy number, leading to …
[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 …
respiratory chain. COX is a multimeric enzyme formed by subunits of dual genetic origin …
A mutant mitochondrial respiratory chain assembly protein causes complex III deficiency in patients with tubulopathy, encephalopathy and liver failure
P De Lonlay, I Valnot, A Barrientos, M Gorbatyuk… - Nature …, 2001 - nature.com
Complex III (CIII; ubiquinol cytochrome c reductase of the mitochondrial respiratory chain)
catalyzes electron transfer from succinate and nicotinamide adenine dinucleotide-linked …
catalyzes electron transfer from succinate and nicotinamide adenine dinucleotide-linked …
Mutations of the SCO1 gene in mitochondrial cytochrome c oxidase deficiency with neonatal-onset hepatic failure and encephalopathy
I Valnot, S Osmond, N Gigarel, B Mehaye… - The American Journal of …, 2000 - cell.com
Cytochrome c oxidase (COX) catalyzes both electron transfer from cytochrome c to
molecular oxygen and the concomitant vectorial proton pumping across the inner …
molecular oxygen and the concomitant vectorial proton pumping across the inner …