Results 11 to 20 of about 60,467 (293)

OXPHOS mutations and neurodegeneration [PDF]

The EMBO Journal, 2012
Mitochondrial oxidative phosphorylation (OXPHOS) sustains organelle function and plays a central role in cellular energy metabolism. The OXPHOS system consists of 5 multisubunit complexes (CI-CV) that are built up of 92 different structural proteins encoded by the nuclear (nDNA) and mitochondrial DNA (mtDNA).
Koopman, W.J.H., Distelmaier, F., Smeitink, J.A.M., Willems, P.H.G.M.   +3 more
openaire   +3 more sources

Mitochondrial disorders of the OXPHOS system [PDF]

FEBS Letters, 2020
Mitochondrial disorders are among the most frequent inborn errors of metabolism, their primary cause being the dysfunction of the oxidative phosphorylation system (OXPHOS). OXPHOS is composed of the electron transport chain (ETC), formed by four multimeric enzymes and two mobile electron carriers, plus an ATP synthase [also called complex V (cV)].
Fernandez-Vizarra, Erika, Zeviani, Massimo   +1 more
openaire   +3 more sources

Secondary coenzyme Q 10 deficiencies in oxidative phosphorylation (OXPHOS) and non-OXPHOS disorders

Mitochondrion, 2016
We evaluated the coenzyme Q₁₀ (CoQ) levels in patients who were diagnosed with mitochondrial oxidative phosphorylation (OXPHOS) and non-OXPHOS disorders (n=72). Data from the 72 cases in this study revealed that 44.4% of patients showed low CoQ concentrations in either their skeletal muscle or skin fibroblasts.
Yubero, D., Montero, R., Martín, M. A., Montoya, J., Ribes, A., Grazina, M., Trevisson, E., Rodriguez-Aguilera, J., Hargreaves, I. P., Salviati, L., Navas, P., Artuch, R., Jou, C., Jimenez-Mallebrera, C., Nascimento, A., Pérez-Dueñas, B., Ortez, C., Ramos, F., Colomer, J., O''Callaghan, M., Pineda, M., García-Cazorla, A., Espinós, C., Ruiz, A., Macaya, A., Marcé-Grau, A., Garcia-Villoria, J., Arias, A., Emperador, S., Ruiz-Pesini, E., Lopez-Gallardo, E., Neergheen, V., Simões, M., Diogo, L., Blázquez, A., González-Quintana, A., Delmiro, A., Domínguez-González, C., Arenas, J., García-Silva, M. T., Martín, E., Quijada, P., Hernández-Laín, A., Morán, M., Rivas Infante, E., Ávila Polo, R., Paradas Lópe, C., Bautista Lorite, J., Martínez Fernández, E. M., Cortés, A. B., Sánchez-Cuesta, A., Cascajo, M. V., Alcázar, M., Brea-Calvo, G.   +53 more
openaire   +7 more sources

ILF2 cooperates with E2F1 to maintain mitochondrial homeostasis and promote small cell lung cancer progression

Cancer Biology & Medicine, 2019
Objective Mitochondria play multifunctional roles in carcinogenesis. Deciphering uncertainties of molecular interactions within mitochondria will promote further understanding of cancer.
Meng Zhao, Yahui Liu, Jiao Chang, Jin Qi, Ran Liu, Yongwang Hou, Yanhui Wang, Xinwei Zhang, Lu Qiao, Li Ren   +9 more
doaj   +1 more source

Chronic Progressive External Ophthalmoplegia Is Associated with a Novel Mutation in the Mitochondrial tRNA(Asn) Gene [PDF]

, 1994
Chronic progressive external ophthalmoplegia (CPEO) is caused by a decreased oxidative phosphorylation (OXPHOS) activity due to large-scale deletions of the mitochondrial genome in 50 % of the patients.
Kadenbach, B., Klopstock, Thomas, Lauber, J., Marsac, C., Reichmann, Heinz, Seibel, P.   +5 more
core   +1 more source

OXPHOS-targeting drugs in oncology: new perspectives

Expert Opinion on Therapeutic Targets, 2023
Drugs targeting mitochondria are emerging as promising antitumor therapeutics in preclinical models. However, a few of these drugs have shown clinical toxicity. Developing mitochondria-targeted modified natural compounds and US FDA-approved drugs with increased therapeutic index in cancer is discussed as an alternative strategy.Triphenylphosphonium ...
Kalyanaraman, Balaraman, Cheng, Gang, Hardy, Micael, You, Ming   +3 more
openaire   +3 more sources

Cardiosphere-derived cells demonstrate metabolic flexibility that Is influenced by adhesion status [PDF]

, 2017
Adult stem cells demonstrate metabolic flexibility that is regulated by cell adhesion status. The authors demonstrate that adherent cells primarily utilize glycolysis, whereas suspended cells rely on oxidative phosphorylation for their ATP needs.
Abraham, M. Roselle, Afzal, Junaid, Aon, Miguel A, Chan, Angel, Foster, D. Brian, Guan, Yufan, Karakas, Mehmet Fatih, O\u27Rourke, Brian, Pomper, Martin, Rathmell, Jeffrey, Tsui, Benjamin, Vakrou, Styliani, Wahl, Richard, Woldemichael, Kirubel   +13 more
core   +2 more sources

Cytochrome c Oxidase Subunit 4 Isoform Exchange Results in Modulation of Oxygen Affinity

Cells, 2020
Cytochrome c oxidase (COX) is regulated through tissue-, development- or environment-controlled expression of subunit isoforms. The COX4 subunit is thought to optimize respiratory chain function according to oxygen-controlled expression of its isoforms ...
David Pajuelo Reguera, Kristýna Čunátová, Marek Vrbacký, Alena Pecinová, Josef Houštěk, Tomáš Mráček, Petr Pecina   +6 more
doaj   +1 more source

Macromolecular crowding explains overflow metabolism in cells [PDF]

, 2016
Overflow metabolism is a metabolic phenotype of cells characterized by mixed oxidative phosphorylation (OxPhos) and fermentative glycolysis in the presence of oxygen.
Oltvai, Zoltán N., Vazquez, Alexei
core   +1 more source

Severe hepatopathy and neurological deterioration after start of valproate treatment in a 6-year-old child with mitochondrial tryptophanyl-tRNA synthetase deficiency [PDF]

, 2018
Background: The first subjects with deficiency of mitochondrial tryptophanyl-tRNA synthetase (WARS2) were reported in 2017. Their clinical characteristics can be subdivided into three phenotypes (neonatal phenotype, severe infantile onset phenotype ...
De Bruyne, Ruth, Menten, Björn, Roels, Frank, Roeyers, Herbert, Smet, Joél, Stepman, Hedwig, Van Coster, Rudy, Vanlander, Arnaud, Vantroys, Elise, Vergult, Sarah   +9 more
core   +1 more source