Results 41 to 50 of about 66,093 (301)

Interference With Complex IV as a Model of Age-Related Decline in Synaptic Connectivity

Frontiers in Molecular Neuroscience, 2020
Age-related impairment of mitochondrial function may negatively impact energy-demanding processes such as synaptic transmission thereby triggering cognitive decline and processes of neurodegeneration.
Martin Kriebel, Julia Ebel, Florian Battke, Stefan Griesbach, Hansjürgen Volkmer   +4 more
doaj   +1 more source

Upregulation of Mitochondrial Content in Cytochrome c Oxidase Deficient Fibroblasts. [PDF]

PLoS ONE, 2016
Cytochrome-c-oxidase (COX) deficiency is a frequent cause of mitochondrial disease and is associated with a wide spectrum of clinical phenotypes. We studied mitochondrial function and biogenesis in fibroblasts derived from the Cohen (CDs) rat, an animal ...
Aviram Kogot-Levin, Ann Saada, Gil Leibowitz, Devorah Soiferman, Liza Douiev, Itamar Raz, Sarah Weksler-Zangen   +6 more
doaj   +1 more source

Siblings with Ethylmalonic Encephalopathy: Case Report

Journal of Pediatric Research, 2018
Deficiency of mitochondrial sulfur dioxygenase (ETHE1) causes a rare inborn error of metabolism, ethylmalonic encephalopathy, which is characterized by early-onset encephalopathy, chronic hemorrhagic diarrhea, recurrent petechiae, orthostatic ...
Çiğdem Seher Kasapkara, Ayşe Aksoy, Emine Polat, Mustafa Kılıç, Serdar Ceylaner   +4 more
doaj   +1 more source

The small protein CydX is required for function of cytochrome bd oxidase in Brucella abortus. [PDF]

, 2012
A large number of hypothetical genes potentially encoding small proteins of unknown function are annotated in the Brucella abortus genome. Individual deletion of 30 of these genes identified four mutants, in BAB1_0355, BAB2_0726, BAB2_0470, and BAB2_0450
de Jong, Maarten F, den Hartigh, Andreas B, Rolán, Hortensia G, Roux, Christelle M, Sun, Yao-Hui, Tsolis, Renée M   +5 more
core   +3 more sources

Murine cytomegalovirus infection exacerbates complex IV deficiency in a model of mitochondrial disease.

PLoS Genetics, 2020
The influence of environmental insults on the onset and progression of mitochondrial diseases is unknown. To evaluate the effects of infection on mitochondrial disease we used a mouse model of Leigh Syndrome, where a missense mutation in the Taco1 gene ...
Nicola Ferreira, Christopher E Andoniou, Kara L Perks, Judith A Ermer, Danielle L Rudler, Giulia Rossetti, Ambika Periyakaruppiah, Jamie K Y Wong, Oliver Rackham, Peter G Noakes, Mariapia A Degli-Esposti, Aleksandra Filipovska   +11 more
doaj   +1 more source

Flux control of cytochrome c oxidase in human skeletal muscle [PDF]

, 2000
In the present work, by titrating cytochrome c oxidase (COX) with the specific inhibitor KCN, the flux control coefficient and the metabolic reserve capacity of COX have been determined in human saponin-permeabilized muscle fibers. In the presence of the
Attardi, Giuseppe, Elger, Christian E., Kudin, Alexei, Kunz, Wolfram S., Vielhaber, Stefan, Villani, Gaetano   +5 more
core   +1 more source

Proteogenomic characterization of cholangiocarcinoma

Hepatology, EarlyView., 2022
Proteogenomic characterization of cholangiocarcinoma with therapeutic strategies Abstract Background and Aims Cholangiocarcinoma (CCA) is a highly heterogeneous cancer with limited understanding and few effective therapeutic approaches. We aimed at providing a proteogenomic CCA characterization to inform biological processes and treatment ...
Mengjie Deng, Peng Ran, Lingli Chen, Yunzhi Wang, Zixiang Yu, Ke Cai, Jinwen Feng, Zhaoyu Qin, Yanan Yin, Subei Tan, Yang Liu, Chen Xu, Guoming Shi, Yuan Ji, Jian‐Yuan Zhao, Jian Zhou, Jia Fan, Yingyong Hou, Chen Ding   +18 more
wiley   +1 more source

NDUFA4 Mutations Underlie Dysfunction of a Cytochrome c Oxidase Subunit Linked to Human Neurological Disease

Cell Reports, 2013
The molecular basis of cytochrome c oxidase (COX, complex IV) deficiency remains genetically undetermined in many cases. Homozygosity mapping and whole-exome sequencing were performed in a consanguineous pedigree with isolated COX deficiency linked to a ...
Robert D.S. Pitceathly, Shamima Rahman, Yehani Wedatilake, James M. Polke, Sebahattin Cirak, A. Reghan Foley, Anna Sailer, Matthew E. Hurles, Jim Stalker, Iain Hargreaves, Cathy E. Woodward, Mary G. Sweeney, Francesco Muntoni, Henry Houlden, Jan-Willem Taanman, Michael G. Hanna   +15 more
doaj   +1 more source

A crucial role of the mitochondrial protein import receptor MOM19 for the biogenesis of mitochondria [PDF]

, 1994
The novel genetic method of "sheltered RIP" (repeat induced point mutation) was used to generate a Neurospora crassa mutant in which MOM19, a component of the protein import machinery of the mitochondrial outer membrane, can be depleted.
Harkness, Troy A. A., Klei, Ida J. van der, Lill, Roland, Nargang, Frank E., Neupert, Walter   +4 more
core   +8 more sources

Essential lipid autacoids rewire mitochondrial energy efficiency in metabolic dysfunction‐associated fatty liver disease

Hepatology, EarlyView., 2022
Increased liver content of DHA‐derived small lipid autacoids (i.e resolvin D1 and maresin 1) associates with enhanced mitochondrial oxidative phosphorylation, fatty acid β‐oxidation and bioenergetic metabolic flux. These features provide hepatic protection from steatotic, pro‐inflammatory and fibrogenic insults.
Cristina López‐Vicario, David Sebastián, Mireia Casulleras, Marta Duran‐Güell, Roger Flores‐Costa, Ferran Aguilar, Juan José Lozano, Ingrid W. Zhang, Esther Titos, Jing X. Kang, Antonio Zorzano, Makoto Arita, Joan Clària   +12 more
wiley   +1 more source