Disease: Coenzyme Q cytochrome c reductase deficiency of
- <em>BCS1</em>Neonatal growth retardation and lactic acidosis initiated by novel mutation sites in <em>L</em> gene
- 4E-BP1 counteracts human mesenchymal stem cell senescence via maintaining mitochondrial homeostasis
- A Defect in Mitochondrial Complex III but Not in Complexes I or IV Causes Early beta-Cell Dysfunction and Hyperglycemia in Mice
- A Defect in Mitochondrial Complex III but Not in Complexes I or IV Causes Early β-Cell Dysfunction and Hyperglycemia in Mice
- A membrane arm of mitochondrial complex I sufficient to promote respirasome formation
- A novel acceptor stem variant in mitochondrial tRNA<sup>Tyr</sup> impairs mitochondrial translation and is associated with a severe phenotype
- A novel mutation in the ubiquinol-cytochrome c reductase synthesis-like gene associated with complex III deficiency and Bjornstad syndrome: A case report
- A Novel Nutraceuticals Mixture Improves Liver Steatosis by Preventing Oxidative Stress and Mitochondrial Dysfunction in a NAFLD Model
- A spontaneous mitonuclear epistasis converging on Rieske Fe-S protein exacerbates complex III deficiency in mice
- A TTC19 mutation associated with progressive movement disorders and peripheral neuropathy: Case report and systematic review
- Acute stress deteriorates breast meat quality of Ross 308 broiler chickens by inducing redox imbalance and mitochondrial dysfunction
- Adult-Onset Deficiency of Mitochondrial Complex III in a Mouse Model of Alzheimer's Disease Decreases Amyloid Beta Plaque Formation
- Alteration of mitochondrial function in the livers of mice with glycogen branching enzyme deficiency
- Anesthetic Hypersensitivity in a Case-Controlled Series of Patients With Mitochondrial Disease
- Ascorbate synthesis as an alternative electron source for mitochondrial respiration: Possible implications for the plant performance
- Autophagy deficiency abolishes liver mitochondrial DNA segregation
- BCS1L mutations produce Fanconi syndrome with developmental disability
- Berberine alleviates lipid metabolism disorders via inhibition of mitochondrial complex I in gut and liver
- Bi-Allelic UQCRFS1 Variants Are Associated with Mitochondrial Complex III Deficiency, Cardiomyopathy, and Alopecia Totalis
- Cavitating leukodystrophy in a case of mitochondrial complex III deficiency due to LYRM7 mutation
- Characterising a homozygous two-exon deletion in UQCRH: comparing human and mouse phenotypes
- Coenzyme Q deficiency in endothelial mitochondria caused by hypoxia; remodeling of the respiratory chain and sensitivity to anoxia/reoxygenation
- Combined Transcriptome and Metabolome Analysis Reveals That the Potent Antifungal Pyrylium Salt Inhibits Mitochondrial Complex I in Candida albicans
- Comparative analysis of mitochondrial genomes provides insights into the mechanisms underlying an S-type cytoplasmic male sterility (CMS) system in wheat (Triticum aestivum L.)
- Comparison of metabolic effects of mitochondrial dysfunctions in the context of vulnerability to fatigue: computer simulation study
- Connexin 43 modulates reverse electron transfer in cardiac mitochondria from inducible knock-out Cx43(Cre-ER(T)/fl) mice by altering the coenzyme Q pool
- Continuous Hypoxia Reduces Retinal Ganglion Cell Degeneration in a Mouse Model of Mitochondrial Optic Neuropathy
- Defective complex III mitochondrial respiratory chain due to a novel variant in CYC1 gene masquerades acute demyelinating syndrome or Leber hereditary optic neuropathy
- Deficiencies in the Mitochondrial Electron Transport Chain Affect Redox Poise and Resistance Toward <em>Colletotrichum higginsianum</em>
- Deficiencies in the Mitochondrial Electron Transport Chain Affect Redox Poise and Resistance Toward Colletotrichum higginsianum
- Disruption of mitochondrial complex III in cap mesenchyme but not in ureteric progenitors results in defective nephrogenesis associated with amino acid deficiency
- Disruption of the Pseudomonas aeruginosa Tat system perturbs PQS-dependent quorum sensing and biofilm maturation through lack of the Rieske cytochrome bc1 sub-unit
- DJ-1 attenuates the glycation of mitochondrial complex I and complex III in the post-ischemic heart
- DMT1 differentially regulates mitochondrial complex activities to reduce glutathione loss and mitigate ferroptosis
- Docosahexaenoic acid increased MeCP2 mediated mitochondrial respiratory complexes II and III enzyme activities in cortical astrocytes
- Duplexing complexome profiling with SILAC to study human respiratory chain assembly defects
- Dysfunctional mitochondrial translation and combined oxidative phosphorylation deficiency in a mouse model of hepatoencephalopathy due to Gfm1 mutations
- Electron transfer flavoprotein and its role in mitochondrial energy metabolism in health and disease
- Evolutionarily conserved susceptibility of the mitochondrial respiratory chain to SDHI pesticides and its consequence on the impact of SDHIs on human cultured cells
- Expanding the phenotypic and molecular spectrum of NFS1-related disorders that cause functional deficiencies in mitochondrial and cytosolic iron-sulfur cluster containing enzymes
- Fasting reveals largely intact systemic lipid mobilization mechanisms in respiratory chain complex III deficient mice
- Fine-tuning of the respiratory complexes stability and supercomplexes assembly in cells defective of complex III
- Genetic heterogeneity and respiratory chain enzyme analysis in pediatric Indian patients with mitochondrial disorder: Report of novel variants in POLG1 gene and their functional implication using molecular dynamic simulation
- Ginsenosides Rb1 and Rg1 Protect Primary Cultured Astrocytes against Oxygen-Glucose Deprivation/Reoxygenation-Induced Injury via Improving Mitochondrial Function
- Global metabolic alterations in colorectal cancer cells during irinotecan-induced DNA replication stress
- Homozygous missense mutation in UQCRC2 associated with severe encephalomyopathy, mitochondrial complex III assembly defect and activation of mitochondrial protein quality control
- Human frataxin, the Friedreich ataxia deficient protein, interacts with mitochondrial respiratory chain
- Hypoxia Promotes Mitochondrial Complex I Abundance via HIF-1alpha in Complex III and Complex IV Eficient Cells
- Hypoxia Promotes Mitochondrial Complex I Abundance via HIF-1α in Complex III and Complex IV Eficient Cells
- Identification of two novel variants of BCS1L gene in a patient with classical GRACILE syndrome
- Inhibition of pyrimidine biosynthesis by strobilurin derivatives induces differentiation of acute myeloid leukemia cells
- Knockout of the Complex III subunit Uqcrh causes bioenergetic impairment and cardiac contractile dysfunction
- LYRM7-associated mitochondrial complex III deficiency with non-cavitating leukoencephalopathy and stroke-like episodes
- Mapping mitochondrial respiratory chain deficiencies by respirometry: Beyond the Mito Stress Test
- MFSD7C switches mitochondrial ATP synthesis to thermogenesis in response to heme
- Mitochondria complex I deficiency in Candida albicans arrests the cell cycle at S phase through suppressive TOR and PKA pathways
- Mitochondrial complex III deficiency drives c-MYC overexpression and illicit cell cycle entry leading to senescence and segmental progeria
- Mitochondrial dysfunction triggers the pathogenesis of Parkinson's disease in neuronal C/EBPβ transgenic mice
- Mitochondrial metabolism is essential for invariant natural killer T cell development and function
- Mitochondrial ubiquinol oxidation is necessary for tumour growth
- Mitoflash generated at the Qo site of mitochondrial Complex III
- Modelling of BCS1L-related human mitochondrial disease in Drosophila melanogaster
- Molecular and Functional Effects of Loss of Cytochrome c Oxidase Subunit 8A
- Molecular pathophysiology of human MICU1 deficiency
- Mthfd2 Modulates Mitochondrial Function and DNA Repair to Maintain the Pluripotency of Mouse Stem Cells
- Multisystem mitochondrial diseases due to mutations in mtDNA-encoded subunits of complex I
- Mutations in the Yeast Cox12 Subunit Severely Compromise the Activity of the Mitochondrial Complex IV
- MYC-an emerging player in mitochondrial diseases
- N-(4-(2-chloro-4-(trifluoromethyl)phenoxy)phenyl)picolinamide as a new inhibitor of mitochondrial complex III: Synthesis, biological evaluation and computational simulations
- Novel pathogenic <em>UQCRC2</em> variants in a female with normal neurodevelopment
- Novel pathogenic UQCRC2 variants in a female with normal neurodevelopment
- Organization of the Respiratory Supercomplexes in Cells with Defective Complex III: Structural Features and Metabolic Consequences
- Oxygen regulation of breathing is abolished in mitochondrial complex III-deficient arterial chemoreceptors
- Pathological Features in Paediatric Patients with TK2 Deficiency
- Pentatricopeptide repeat protein CNS1 regulates maize mitochondrial complex III assembly and seed development
- Photobiomodulation therapy ameliorates hyperglycemia and insulin resistance by activating cytochrome c oxidase-mediated protein kinase B in muscle
- Progressive cerebellar atrophy in a patient with complex II and III deficiency and a novel deleterious variant in SDHA: A Counseling Conundrum
- Reactive oxygen species production by BP-1,6-quinone and its effects on the endothelial dysfunction: Involvement of the mitochondria
- Respiratory supercomplexes act as a platform for complex III-mediated maturation of human mitochondrial complexes I and IV
- SDHA gain-of-function engages inflammatory mitochondrial retrograde signaling via KEAP1-Nrf2
- Simultaneously autotrophic denitrification and organics degradation in low-strength coal gasification wastewater (LSCGW) treatment via microelectrolysis-triggered Fe(II)/Fe(III) cycle
- Superoxide produced by mitochondrial complex III plays a pivotal role in the execution of ferroptosis induced by cysteine starvation
- The cardiac-enriched microprotein mitolamban regulates mitochondrial respiratory complex assembly and function in mice
- The complex II resistance mutation H258Y in succinate dehydrogenase subunit B causes fitness penalties associated with mitochondrial respiratory deficiency
- The DYW-subgroup pentatricopeptide repeat protein PPR27 interacts with ZmMORF1 to facilitate mitochondrial RNA editing and seed development in maize
- The Effect of Deflazacort Treatment on the Functioning of Skeletal Muscle Mitochondria in Duchenne Muscular Dystrophy
- The Effect of Methylmalonic Acid Treatment on Human Neuronal Cell Coenzyme Q<sub>10</sub> Status and Mitochondrial Function
- The Effect of Neuronal CoQ(10) Deficiency and Mitochondrial Dysfunction on a Rotenone-Induced Neuronal Cell Model of Parkinson's Disease
- The Effect of Organophosphate Exposure on Neuronal Cell Coenzyme Q<sub>10</sub> Status
- The mitochondrial carrier SFXN1 is critical for complex III integrity and cellular metabolism
- The Novel J-Domain Protein Mrj1 Is Required for Mitochondrial Respiration and Virulence in Cryptococcus neoformans
- The striking differences in the bioenergetics of brain and liver mitochondria are enhanced in mitochondrial disease
- Total and reduced/oxidized forms of coenzyme Q<sub>10</sub> in fibroblasts of patients with mitochondrial disease
- Trialkylsilyl-Substituted Silole and Germole Dianions as Precursors for Unusual Silicon and Germanium Compounds
- Trib1 deficiency causes brown adipose respiratory chain depletion and mitochondrial disorder
- Ubiquinol-cytochrome c reductase core protein 1 overexpression protects H9c2 cardiac cells against mimic ischemia/reperfusion injury through PI3K/Akt/GSK-3β pathway
- Unveiling a key role of oxaloacetate-glutamate interaction in regulation of respiration and ROS generation in nonsynaptic brain mitochondria using a kinetic model
- UQCRC1 engages cytochrome c for neuronal apoptotic cell death
- UQCRC2-related mitochondrial complex III deficiency, about 7 patients
- Vitamin C modulates the levels of several proteins of the mitochondrial complex III and its activity in the mouse liver