Results 11 to 20 of about 46,956 (278)

OXPHOS mutations and neurodegeneration [PDF]

EMBO Journal, 2013
Contains fulltext : 118892.pdf (Publisher’s version ) (Open Access)Mitochondrial oxidative phosphorylation (OXPHOS) sustains organelle function and plays a central role in cellular energy metabolism.
Werner J H Koopman, Felix Distelmaier, Peter H G M Willems   +2 more
exaly   +6 more sources

In vivo and in organello assessment of OXPHOS activities

Methods, 2002
Mitochondrial OXPHOS defects are responsible for a large group of human diseases and have been associated with degenerative disorders and aging. The accurate in vivo and in organello biochemical assessment of the OXPHOS system is necessary for the ...
Antoni Barrientos
exaly   +4 more sources

Altered Expression Ratio of Actin-Binding Gelsolin Isoforms Is a Novel Hallmark of Mitochondrial OXPHOS Dysfunction [PDF]

Cells, 2020
Mitochondrial oxidative phosphorylation (OXPHOS) defects are the primary cause of inborn errors of energy metabolism. Despite considerable progress on their genetic basis, their global pathophysiological consequences remain undefined.
Ana Penas, Maria Illescas, Rafael Perez-Perez   +2 more
exaly   +4 more sources

Coordinating mitochondrial translation with assembly of the OXPHOS complexes

Human Molecular Genetics
The mitochondrial oxidative phosphorylation (OXPHOS) system produces the majority of energy required by cells. Given the mitochondrion’s endosymbiotic origin, the OXPHOS machinery is still under dual genetic control where most OXPHOS subunits are encoded
Peter Rehling, Rehling Peter
exaly   +7 more sources

Secondary coenzyme Q10 deficiencies in oxidative phosphorylation (OXPHOS) and non-OXPHOS disorders [PDF]

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).
Cortés, A. B., García-Cazorla, A., Ruiz, A., Espinós, C., Ávila Polo, R., Pérez-Dueñas, B., Rodriguez-Aguilera, J., Montoya, J., Nascimento, A., Alcázar, M., Jou, C., Artuch, R., Simões, M., González-Quintana, A., Macaya, A., Grazina, M., Sánchez-Cuesta, A., Pineda, M., Emperador, S., Rivas Infante, E., Brea-Calvo, G., Lopez-Gallardo, E., Martín, E., Paradas Lópe, C., Jimenez-Mallebrera, C., Morán, M., Martínez Fernández, E. M., Marcé-Grau, A., Blázquez, A., Yubero, D., Montero, R., Garcia-Villoria, J., Neergheen, V., Martín, M. A., O''Callaghan, M., Trevisson, E., Arenas, J., Ortez, C., Bautista Lorite, J., Delmiro, A., Hargreaves, I. P., Ruiz-Pesini, E., Arias, A., Ramos, F., Colomer, J., Quijada, P., Cascajo, M. V., García-Silva, M. T., Ribes, A., Salviati, L., Diogo, L., Domínguez-González, C., Navas, P., Hernández-Laín, A.   +53 more
core   +10 more sources

Genetic defects in the oxidative phosphorylation (OXPHOS) system

Expert Review of Molecular Diagnostics, 2004
Item does not contain fulltextThe oxidative phosphorylation (OXPHOS) system consists of five multiprotein complexes and two mobile electron carriers embedded in the lipid bilayer of the mitochondrial inner membrane.
L P Van den Heuvel
exaly   +5 more sources

Targeting Mitochondrial OXPHOS and Their Regulatory Signals in Prostate Cancers

International Journal of Molecular Sciences, 2021
Increasing evidence suggests that tumor development requires not only oncogene/tumor suppressor mutations to drive the growth, survival, and metastasis but also metabolic adaptations to meet the increasing energy demand for rapid cellular expansion and ...
Chia-Lin Chen, Ching-Yu Lin, Lin Ching-Yu   +2 more
exaly   +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).
Fernandez-Vizarra, Erika, Erika Fernandez‐Vizarra, Massimo Zeviani, Zeviani, Massimo   +3 more
core   +4 more sources

The molecular evolutionary dynamics of oxidative phosphorylation (OXPHOS) genes in Hymenoptera [PDF]

BMC Evolutionary Biology, 2017
Background The primary energy-producing pathway in eukaryotic cells, the oxidative phosphorylation (OXPHOS) system, comprises proteins encoded by both mitochondrial and nuclear genes.
Yiyuan Li, Rui Zhang, Shanlin Liu, Alexander Donath, Ralph S. Peters, Jessica Ware, Bernhard Misof, Oliver Niehuis, Michael E. Pfrender, Xin Zhou   +9 more
doaj   +2 more sources

OXPHOS toxicogenomics and Parkinson's disease

Mutation Research/Reviews in Mutation Research, 2011
Activities and quantities of several oxidative phosphorylation (OXPHOS) system complexes are decreased in many Parkinson's disease (PD) patients. Mutations in PD-associated nuclear genes affect OXPHOS function. Moreover, the inactivation of other nuclear
Eldris Iglesias, Eduardo Ruiz-Pesini, Ester López-Gallardo, Ruth Iceta, López-Gallardo,E., Montoya,J., Ruiz-Pesini,E., Iceta,R., Iglesias,E., Julio Montoya   +9 more
core   +4 more sources