Results 11 to 20 of about 51,036 (282)

Secondary coenzyme Q 10 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). 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
core   +7 more sources

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   +6 more sources

Bayesian classification of OXPHOS deficient skeletal myofibres [PDF]

PLOS Computational Biology
Mitochondria are organelles in most human cells which release the energy required for cells to function. Oxidative phosphorylation (OXPHOS) is a key biochemical process within mitochondria required for energy production and requires a range of proteins and protein complexes.
Jordan Childs, Tiago Bernardino Gomes, Amy E. Vincent, Andrew Golightly, Conor Lawless   +4 more
core   +11 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   +5 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 genes related to mitochondrial DNA (mtDNA) replication and expression also leads to Parkinsonism ...
López-Gallardo,E., Iceta,R., Iglesias,E., Montoya,J., Ruiz-Pesini,E.   +4 more
openaire   +4 more sources

Proteinopathies and OXPHOS dysfunction in neurodegenerative diseases [PDF]

Journal of Cell Biology, 2017
Mitochondria participate in essential processes in the nervous system such as energy and intermediate metabolism, calcium homeostasis, and apoptosis. Major neurodegenerative diseases are characterized pathologically by accumulation of misfolded proteins as a result of gene mutations or abnormal protein homeostasis.
Hibiki Kawamata, Giovanni Manfredi
openaire   +3 more sources

Mitoribosomal regulation of OXPHOS biogenesis in plants [PDF]

Frontiers in Plant Science, 2014
The ribosome filter hypothesis posits that ribosomes are not simple non-selective translation machines but may also function as regulatory elements in protein synthesis. Recent data supporting ribosomal filtering come from plant mitochondria where it has been shown that translation of mitochondrial transcripts encoding components of oxidative ...
Janska, Hanna, Kwasniak, Malgorzata
openaire   +5 more sources

Crif1 deficiency reduces adipose OXPHOS capacity and triggers inflammation and insulin resistance in mice. [PDF]

PLoS Genetics, 2013
Impaired mitochondrial oxidative phosphorylation (OXPHOS) has been proposed as an etiological mechanism underlying insulin resistance. However, the initiating organ of OXPHOS dysfunction during the development of systemic insulin resistance has yet to be
Min Jeong Ryu, Soung Jung Kim, Yong Kyung Kim, Min Jeong Choi, Surendar Tadi, Min Hee Lee, Seong Eun Lee, Hyo Kyun Chung, Saet Byel Jung, Hyun-Jin Kim, Young Suk Jo, Koon Soon Kim, Sang-Hee Lee, Jin Man Kim, Gi Ryang Kweon, Ki Cheol Park, Jung Uee Lee, Young Yun Kong, Chul-Ho Lee, Jongkyeong Chung, Minho Shong   +20 more
doaj   +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-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   +4 more sources