Mosaic origin of the mitochondrial proteome

R Szklarczyk, MA Huynen - Proteomics, 2010 - Wiley Online Library
Although the origin of mitochondria from the endosymbiosis of an α‐proteobacterium is well
established, the nature of the host cell, the metabolic complexity of the endosymbiont and …

[PDF][PDF] NDUFA4 is a subunit of complex IV of the mammalian electron transport chain

E Balsa, R Marco, E Perales-Clemente, R Szklarczyk… - Cell metabolism, 2012 - cell.com
… MtDNA-less mouse cells (r L929) were grown in DMEM supplemented with 5% FBS, 50
mg/ml uridine, and 1 mM pyruvate (r medium) and in the presence of 250 mg/ml of geneticin (…

How to deal with oxygen radicals stemming from mitochondrial fatty acid oxidation

…, GR Manjeri, R Szklarczyk - … Transactions of the …, 2014 - royalsocietypublishing.org
Oxygen radical formation in mitochondria is an incompletely understood attribute of
eukaryotic cells. Recently, a kinetic model was proposed, in which the ratio between electrons …

Control of mitochondrial integrity in ageing and disease

R Szklarczyk, M Nooteboom… - … Transactions of the …, 2014 - royalsocietypublishing.org
… This creates two forms of methionine sulfoxide, S and R. The two diastereomers can be
reduced by the peptide methionine sulfoxide reductases A (MsrA) and B (Msr B). Both sulfoxide …

[PDF][PDF] CLPB mutations cause 3-methylglutaconic aciduria, progressive brain atrophy, intellectual disability, congenital neutropenia, cataracts, movement disorder

…, S Ziętkiewicz, M Kousi, R Szklarczyk… - The American Journal of …, 2015 - cell.com
We studied a group of individuals with elevated urinary excretion of 3-methylglutaconic acid,
neutropenia that can develop into leukemia, a neurological phenotype ranging from …

[HTML][HTML] Mutations in the UQCC1-Interacting Protein, UQCC2, Cause Human Complex III Deficiency Associated with Perturbed Cytochrome b Protein Expression

EJ Tucker, BFJ Wanschers, R Szklarczyk… - PLoS …, 2013 - journals.plos.org
Mitochondrial oxidative phosphorylation (OXPHOS) is responsible for generating the majority
of cellular ATP. Complex III (ubiquinol-cytochrome c oxidoreductase) is the third of five …

Tracking repeats using significance and transitivty.

R Szklarczyk, J Heringa - Bioinformatics, 2004 - research.vu.nl
Szklarczyk and J.Heringa … Having scorew and jmax calculated, we can obtain the
relative contribution of a residue r in the k-th column of the profile prel(r, k) = …

[HTML][HTML] CiliaCarta: An integrated and validated compendium of ciliary genes

…, M Schmidts, PL Beales, Q Lu, RH Giles, R Szklarczyk… - PloS one, 2019 - journals.plos.org
The cilium is an essential organelle at the surface of mammalian cells whose dysfunction
causes a wide range of genetic diseases collectively called ciliopathies. The current rate at …

[HTML][HTML] Whole exome sequencing is the preferred strategy to identify the genetic defect in patients with a probable or possible mitochondrial cause

…, M De Visser, J Vanoevelen, R Szklarczyk… - Frontiers in …, 2018 - frontiersin.org
Mitochondrial disorders, characterized by clinical symptoms and/or OXPHOS deficiencies,
are caused by pathogenic variants in mitochondrial genes. However, pathogenic variants in …

[HTML][HTML] Iterative orthology prediction uncovers new mitochondrial proteins and identifies C12orf62 as the human ortholog of COX14, a protein involved in the …

R Szklarczyk, BFJ Wanschers, TD Cuypers, JJ Esseling… - Genome biology, 2012 - Springer
Background Orthology is a central tenet of comparative genomics and ortholog identification
is instrumental to protein function prediction. Major advances have been made to determine …