Disease: Complex 1 mitochondrial respiratory chain deficiency
- A Drosophila model of mitochondrial disease phenotypic heterogeneity
- A novel mitochondrial DNA variant in MT-ND6: m.14430A>C p.(Trp82Gly) identified in a patient with Leigh syndrome and complex I deficiency
- A novel mouse model of mitochondrial disease exhibits juvenile-onset severe neurological impairment due to parvalbumin cell mitochondrial dysfunction
- A reversible state of hypometabolism in a human cellular model of sporadic Parkinson's disease
- Aggregated chromosomes/chromatin transfer: a novel approach for mitochondrial replacement with minimal mitochondrial carryover-the implications of mouse experiments for human aggregated chromosome transfer
- Aggregated chromosomes/chromatin transfer: a novel approach for mitochondrial replacement with minimal mitochondrial carryover: the implications of mouse experiments for human aggregated chromosome transfer
- Alternative oxidase blunts pseudohypoxia and photoreceptor degeneration due to RPE mitochondrial dysfunction
- Analyzing Mitochondrial Function in a Drosophila melanogaster PINK1B9-Null Mutant Using High-resolution Respirometry
- Autophagy core protein BECN1 is vital for spermatogenesis and male fertility in mice
- Bi-allelic ACBD6 variants lead to a neurodevelopmental syndrome with progressive and complex movement disorders
- Biallelic NDUFA4 Deletion Causes Mitochondrial Complex IV Deficiency in a Patient with Leigh Syndrome
- Cardioprotective Effect of 2-Ethyl-3-Hydroxy-6-Methylpyridinium 2-Nitroxysuccinate Against Adrenaline/Hydrocortisone-Induced Myocardial Ischemia in Mice: Modulation of Free-Radical Processes in Biomembranes and Monoamine Oxidase A Activity
- Challenges in Genetic Diagnosis of Mitochondrial Diseases: What Can Functional Genomics' Studies Do?
- CISD3/MiNT is required for complex I function, mitochondrial integrity, and skeletal muscle maintenance
- CKLF induces microglial activation via triggering defective mitophagy and mitochondrial dysfunction
- Co-fractionation-mass spectrometry to characterize native mitochondrial protein assemblies in mammalian neurons and brain
- Complex I, V, and MDH2 deficient human skin fibroblasts reveal distinct metabolic signatures by (1) H HR-MAS NMR
- Complex mitochondrial disease caused by the mutation of COX10 in a toddler: a case-report study
- COX17 acetylation via MOF-KANSL complex promotes mitochondrial integrity and function
- COX6C expression driven by copy amplification of 8q22.2 regulates cell proliferation via mediation of mitosis by ROS-AMPK signaling in lung adenocarcinoma
- Crosstalk between mitochondrial biogenesis and mitophagy to maintain mitochondrial homeostasis
- Cryo-electron tomography of NLRP3-activated ASC complexes reveals organelle co-localization
- Curcumin-ZnO conjugated nanoparticles confer neuroprotection against ketamine-induced neurotoxicity
- Cytochrome c oxidase IV isoform 1 (COX4-1) regulates the proliferation, migration and invasion of trophoblast cells via modulating mitochondrial function
- Decoding metabolic signatures in Alzheimer's disease: a mitochondrial perspective
- Defective mitochondrial COX1 translation due to loss of COX14 function triggers ROS-induced inflammation in mouse liver
- Directed proton transfer from F<sub>o</sub> to F<sub>1</sub> extends the multifaceted proton functions in ATP synthase
- Disrupted brain mitochondrial morphology after in vivo hydrogen sulfide exposure
- Diversities in Leigh Syndrome Associated with MT-ATP6 Gene Variants
- Down-regulation of Lon protease 1 lysine crotonylation aggravates mitochondrial dysfunction in polycystic ovary syndrome
- Effect of CB1 Receptor Deficiency on Mitochondrial Quality Control Pathways in Gastrocnemius Muscle
- Effects of Microplastic (MP) Exposure at Environmentally Relevant Doses on the Structure, Function, and Transcriptome of the Kidney in Mice
- Enhancing Nix-dependent mitophagy relieves AKI by restricting TREM-1-mediated hyperactivation of inflammasome in platelets
- Expanding the genetic and phenotypic spectrum of Baker-Gordon syndrome: a new <em>de novo SYT1</em> variant
- Functions of the primary cilium in the kidney and its connection with renal diseases
- Fut2 Deficiency Promotes Intestinal Stem Cell Aging by Damaging Mitochondrial Functions via Down-Regulating alpha1,2-Fucosylation of Asah2 and Npc1
- GCN5L1 regulates pulmonary surfactant production by modulating lamellar body biogenesis and trafficking in mouse alveolar epithelial cells
- Genetic and bioinformatic analyses reveal transcriptional networks underlying dual genomic coordination of mitochondrial biogenesis
- Genetic and epigenetic targets of natural dietary compounds as anti-Alzheimer's agents
- 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
- Genetic, metabolic and clinical delineation of an MRPS23-associated mitochondrial disorder
- GLUT-1 changes in paediatric Huntington disease brain cortex and fibroblasts: an observational case-control study
- Hypertrophic cardiomyopathy is characterized by alterations of the mitochondrial calcium uniporter complex proteins: insights from patients with aortic valve stenosis versus hypertrophic obstructive cardiomyopathy
- IARS2 mutations lead to Leigh syndrome with a combined oxidative phosphorylation deficiency
- In fission yeast, 65 non-essential mitochondrial proteins related to respiration and stress become essential in low-glucose conditions
- In vivo imaging of mitochondrial DNA mutations using an integrated nano Cas12a sensor
- Increased Diagnostic Yield by Reanalysis of Whole Exome Sequencing Data in Mitochondrial Disease
- Induced pluripotent stem cells derived from patients carrying mitochondrial mutations exhibit altered bioenergetics and aberrant differentiation potential
- Inhibition of PDGFRβ alleviates endothelial cell apoptotic injury caused by DRP-1 overexpression and mitochondria fusion failure after mitophagy
- Lack of mitochondrial complex I assembly factor NDUFAF2 results in a distinctive infantile-onset brainstem neurodegenerative disease with early lethality
- Living donor liver transplantation for myocerebrohepatopathy spectrum due to POLG mutations
- LncRNAs are involved in regulating ageing and age-related disease through the adenosine monophosphate-activated protein kinase signalling pathway
- Longitudinal Analysis of Mitochondrial Function in a Choline-Deficient L-Amino Acid-Defined High-Fat Diet-Induced Metabolic Dysfunction-Associated Steatohepatitis Mouse Model
- MCCC2 is a novel mediator between mitochondria and telomere and functions as an oncogene in colorectal cancer
- Mitochondria: one of the vital hubs for molecular hydrogen's biological functions
- Mitochondrial complex I deficiency stratifies idiopathic Parkinson's disease
- Mitochondrial complex I subunit NDUFS8.2 modulates responses to stresses associated with reduced water availability
- Mitochondrial dysfunction abrogates dietary lipid processing in enterocytes
- Mitochondrial dysfunction in Parkinson's disease - a key disease hallmark with therapeutic potential
- Mitochondrial impairment, decreased sirtuin activity and protein acetylation in dorsal root ganglia in Friedreich Ataxia models
- Mitochondrial respiration in microglia is essential for response to demyelinating injury but not proliferation
- Mitochondrial respiration is essential for photosynthesis-dependent ATP supply of the plant cytosol
- Mitochondrial-mediated nuclear remodeling and macrophage polarizations: A key switch from liver fibrosis to HCC progression
- Modulation of mitochondrial function with near-infrared light reduces brain injury in a translational model of cardiac arrest
- Molecular diagnosis of visceral leishmaniasis: a French study comparing a reference PCR method targeting kinetoplast DNA and a commercial kit targeting ribosomal DNA
- Molecular Identification of Aedes, Armigeres, and Culex Mosquitoes (Diptera: Culicidae) Using Mitochondrial Cytochrome Oxidase Subunit I Genes in Myanmar
- Molecular mechanisms underlying NLRP3 inflammasome activation and IL-1β production in air pollution fine particulate matter (PM<sub>2.5</sub>)-primed macrophages
- Multi-level profiling unravels mitochondrial dysfunction in myotonic dystrophy type 2
- Mutation at the entrance of the quinone cavity severely disrupts quinone binding in respiratory complex I
- N-Acetylglutamate and N-acetylmethionine compromise mitochondrial bioenergetics homeostasis and glutamate oxidation in brain of developing rats: Potential implications for the pathogenesis of ACY1 deficiency
- Natriuretic peptide receptor-C perturbs mitochondrial respiration in white adipose tissue
- New insights into the nutritional genomics of adult-onset riboflavin-responsive diseases
- Nicotinamide Riboside Supplementation Restores Myocardial Nicotinamide Adenine Dinucleotide Levels, Improves Survival, and Promotes Protective Environment Post Myocardial Infarction
- NLRP3 inflammasome in cognitive impairment and pharmacological properties of its inhibitors
- Plate-Based Assays for the Characterization of Mitochondrial and Cellular Phenotypes
- Post-Subfunctionalization Functions of HIF-1αA and HIF-1αB in Cyprinid Fish: Fine-Tuning Mitophagy and Apoptosis Regulation Under Hypoxic Stress
- PPTC7 maintains mitochondrial protein content by suppressing receptor-mediated mitophagy
- Prominent muscle involvement in a familial form of mitochondrial disease due to a <em>COA8</em> variant
- Prominent muscle involvement in a familial form of mitochondrial disease due to a COA8 variant
- Promising 8-Aminoquinoline-Based Metal Complexes in the Modulation of SIRT1/3-FOXO3a Axis against Oxidative Damage-Induced Preclinical Neurons
- Regulation of Drp1 and enhancement of mitochondrial fission by the deubiquitinating enzyme PSMD14 facilitates the proliferation of bladder cancer cells
- Research advances on molecular mechanism and natural product therapy of iron metabolism in heart failure
- RICTOR/mTORC2 downregulation in BRAF<sup>V600E</sup> melanoma cells promotes resistance to BRAF/MEK inhibition
- Sheng Mai Yin shows anti-fatigue, anti-hypoxia and cardioprotective potential in an experimental joint model of fatigue and acute myocardial infarction
- SIRT5 rs12216101 T>G variant is associated with liver damage and mitochondrial dysfunction in patients with non-alcoholic fatty liver disease
- Sodium butyrate alleviates R97-116 peptide-induced myasthenia gravis in mice by improving the gut microbiota and modulating immune response
- Structural insights into respiratory complex I deficiency and assembly from the mitochondrial disease-related ndufs4(-/-) mouse
- Structural insights into respiratory complex I deficiency and assembly from the mitochondrial disease-related ndufs4<sup>-/-</sup> mouse
- SUMOylation Modulates Reactive Oxygen Species (ROS) Levels and Acts as a Protective Mechanism in the Type 2 Model of Diabetic Peripheral Neuropathy
- Targeting Mitochondrial Complex I Deficiency in MPP(+)/MPTP-induced Parkinson's Disease Cell Culture and Mouse Models by Transducing Yeast NDI1 Gene
- Targeting PRSS23 with tipranavir induces gastric cancer stem cell apoptosis and inhibits growth of gastric cancer via the MKK3/p38 MAPK-IL24 pathway
- The activator protein-1 complex governs a vascular degenerative transcriptional programme in smooth muscle cells to trigger aortic dissection and rupture
- The impact of aging and oxidative stress in metabolic and nervous system disorders: programmed cell death and molecular signal transduction crosstalk
- The mitochondrial fusion protein OPA1 is dispensable in the liver and its absence induces mitohormesis to protect liver from drug-induced injury
- The mitochondrial protease PARL is required for spermatogenesis
- The rod synapse in aging wildtype and Dscaml1 mutant mice
- The tumor-enriched small molecule gambogic amide suppresses glioma by targeting WDR1-dependent cytoskeleton remodeling
- The ubiquitin-proteasome pathway inhibitor TAK-243 has major effects on calcium handling in mammalian cells
- Therapeutic outcome of patients with Lennox-Gastaut syndrome with mitochondrial respiratory chain complex I deficiency
- Tom70-regulated mitochondrial biogenesis via TFAM improves hypoxia-induced dysfunction of pulmonary vascular endothelial cells and alleviates hypoxic pulmonary hypertension