Mitochondrial respiratory chain complex III
Exome sequencing reveals a novel TTC19 mutation in an autosomal recessive spinocerebellar ataxia patient
... Results: After these manipulations, we identified a homozygous nonsense mutation of the TTC19 gene (p.Q277*). TTC19 has been reported to be a causative gene of a neurodegenerative disease in Italian and Portuguese ...
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Recurrent inhibition of mitochondrial complex III induces chronic pulmonary vasoconstriction and glycolytic switch in the rat lung
... drial respiratory chain disease is accompanied by a mani- festation of primary pulmonary hypertension (PPH) and severe pulmonary hypertension [15, ...the respiratory complexes activity indicated a ...
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Mitochondrial genome variability: the effect on cellular functional activity
... the respiratory chain complexes I and II, ...to complex III in a reduced state in order to be ...re-oxidized. Respiratory chain complex III reduces cytochrome c, ...
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Involvement of an Alternative Oxidase in Oxidative Stress and Mycelium-to-Yeast Differentiation in Paracoccidioides brasiliensis
... of mitochondrial respiratory chain inhibitors on ...drial respiratory chain and alternative ...of complex IV inhibitor KCN (the first chlamydospore-like structure observed after ...
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Nutritional Intervention with Tricarboxylic Acid Cycle Intermediates Modulates Mitochondrial Flux and Respiratory Chain Complex Abundance in C2C12 Myoblast Cells
... of respiratory chain complex and ATPase abundance in mouse mitochondria (Abcam; ab110413) which was specifically detected using a goat anti-mouse horse radishperoxidase labeled secondary antibody ...
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Myocardial Layer Specific Strain Analysis in Children with Mitochondrial Disease
... (complex III), cytochrome c oxidase (complex IV), oligo- mycin-sensitive ATPase (complex V), and citrate synthase activity were also ...tochondrial respiratory chain ...
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Clinical Reasoning: A 58-year-old man with progressive ptosis and walking difficulty
... of mitochondrial respiratory chain enzyme complex I, I 1 III, II 1 III, and ...the respiratory chain, as each complex is composed of subunits encoded by both ...
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Mitochondrial function in flying honeybees (Apis mellifera): respiratory chain enzymes and electron flow from complex III to oxygen
... into mitochondrial function during flight, metabolic rates of individual flying honeybees were measured using respirometry, and their thoracic muscles were fixed for electron ...microscopy. Mitochondrial ...
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Involvement of p32 and Microtubules in Alteration of Mitochondrial Functions by Rubella Virus
... of respiratory chain com- plex III than in the RV-infected solvent ...of mitochondrial activity by ...of respiratory chain complexes, such as the HIV-1- induced increase in ...
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Pioglitazone leads to an inactivation and disassembly of complex I of the mitochondrial respiratory chain
... of respiratory supercomplexes To investigate how PGZ affects the formation of respira- tory supercomplexes, we performed BN-PAGE using the detergent digitonin instead of ...that complex I was present in the ...
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Complex I and energy thresholds in the brain
... transport chain complexes, their supramolecular organization into ‘ respirasomes ’ is relatively an under-studied ...model complex I – IV moves freely by lateral diffusion in the inner mitochondrial ...
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Respiratory chain complex III deficiency due to mutated BCS1L: a novel phenotype with encephalomyopathy, partially phenocopied in a Bcs1l mutant mouse model
... astrogliosis, specifically involving the deeper layers of the cerebral cortex, which could be specific to this severe phenotype (as it transcribes in the animal model) or be a mere result of ATP depletion [27]. An ...
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Studies on the genetics and molecular pathogenesis of mitochondrial respiratory chain disorders
... the complex I in the mitochondrial ...f complex I-associated iron-sulfur clusters [Dupuis et a l 1998;Alves and Videira ...the complex I ...the mitochondrial protein concentrations o f ...
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Neuroprotective Potential of Genistein in Mitochondrial Dysfunction Mediated Apoptosis in Diabetic Neuronal Complications
... compromised mitochondrial function ultimately causes neuronal apoptosis and neurodegeneration causing cognitive dysfunctions and ...maintaining mitochondrial integrity might be highly beneficial in such ...
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Mitochondrial Signaling in Hypoxia
... Mitochondria play a key role in oxygen homeostasis. This role is determined primarily by the trigger function of mitochondria in the cell, the function of a self-regu- lating biological machine that uses oxygen for ...
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Decoding Warburg’s hypothesis: tumor-related mutations in the mitochondrial respiratory chain
... with mitochondrial encephalomyopathy [111], cybrids with one of these mutations were obtained by fusing enucleated fibroblasts from patients to one of these mitochondrial mutations with HeLa cells without ...
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Original Article Overexpression of GRIM-19, a mitochondrial respiratory chain complex I protein, suppresses hepatocellular carcinoma growth
... the mitochondrial apoptotic machinery by inhibiting Bax and Bak oligomer- ization ...into mitochondrial membranes and lead to cytochrome C release and cytotoxic ...inner mitochondrial membranes into ...
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Platelet mitochondrial dysfunction in critically ill patients: comparison between sepsis and cardiogenic shock
... platelet mitochondrial physiology, such as oxygen consumption or membrane potential, were not ...Platelet mitochondrial density was evaluated in terms of citrate synthase activity (normal even in critically ...
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The mitochondria mediate the induction of NOX1 gene expression by aldosterone in an ATF-1-dependent manner
... The A7r5 rat aortic smooth muscle cell line was obtained from the American Type Culture Collection (Rockville, MD). The control solutions contained the appropriate amount of the vehicle, ethanol, for the aldosterone ...
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Combined therapeutic effects of low power laser (980nm) and CoQ10 on Neuropathic Pain in adult male rat
... with mitochondrial dysfunction and lead to increasing ROS production and oxidative stress generation ...in mitochondrial dysfunction. It acts via re- ducing complex III activity, increasing ...
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