Disease: Complex 5 mitochondrial respiratory chain deficiency
- <em>Mtrr</em> hypomorphic mutation alters liver morphology, metabolism and fuel storage in mice
- A deafness-associated mitochondrial DNA mutation caused pleiotropic effects on DNA replication and tRNA metabolism
- A Drosophila model of mitochondrial disease phenotypic heterogeneity
- A meta-analysis and systematic review of Leigh syndrome: clinical manifestations, respiratory chain enzyme complex deficiency, and gene mutations
- 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 variant in the COX15 gene causing a fatal infantile cardioencephalomyopathy: A case report with clinical and molecular review
- A plasma metabolomic signature of Leber hereditary optic neuropathy showing taurine and nicotinamide deficiencies
- A rare case of primary coenzyme Q10 deficiency due to COQ9 mutation
- Aberrant BCAA and glutamate metabolism linked to regional neurodegeneration in a mouse model of Leigh syndrome
- Abnormalities of mitochondrial dynamics and bioenergetics in neuronal cells from CDKL5 deficiency disorder
- Anesthetic Hypersensitivity in a Case-Controlled Series of Patients With Mitochondrial Disease
- Asparagine couples mitochondrial respiration to ATF4 activity and tumor growth
- Autistic feature as a presentation of Inborn Errors of Metabolism
- Autophagic protein ATG5 controls antiviral immunity via glycolytic reprogramming of dendritic cells against respiratory syncytial virus infection
- Biallelic <em>COX10</em> Mutations and <em>PMP22</em> Deletion in a Family With Leigh Syndrome and Hereditary Neuropathy With Liability to Pressure Palsy
- Breathe: Your Mitochondria Will Do the Rest If They Are Healthy!
- Burn-Induced Cardiac Mitochondrial Dysfunction via Interruption of the PDE5A-cGMP-PKG Pathway
- Cardioprotective effects of idebenone do not involve ROS scavenging: Evidence for mitochondrial complex I bypass in ischemia/reperfusion injury
- Case Report: A Variant Non-ketotic Hyperglycinemia With GLRX5 Mutations: Manifestation of Deficiency of Activities of the Respiratory Chain Enzymes
- Case Report: Optic Atrophy and Nephropathy With m.13513G>A/MT-ND5 mtDNA Pathogenic Variant
- Clinical and genetic characteristics of children with COX20-associated mitochondrial disorder: case report and literature review
- Coenzyme Q biochemistry and biosynthesis
- Comparative analysis of mitochondrial genomes provides insights into the mechanisms underlying an S-type cytoplasmic male sterility (CMS) system in wheat (Triticum aestivum L.)
- Cystathionine-gamma-lyase (CSE) deficiency increases erythropoiesis and promotes mitochondrial electron transport via the upregulation of coproporphyrinogen III oxidase and consequent stimulation of heme biosynthesis
- Deafness-associated tRNA(Phe) mutation impaired mitochondrial and cellular integrity
- Detecting respiratory chain defects in osteoblasts from osteoarthritic patients using imaging mass cytometry
- Dichloroacetate and thiamine improve survival and mitochondrial stress in a C. elegans model of dihydrolipoamide dehydrogenase deficiency
- Dietary Iron Deficiency Modulates Adipocyte Iron Homeostasis, Adaptive Thermogenesis, and Obesity in C57BL/6 Mice
- Diverse mitochondrial abnormalities in a new cellular model of TAFFAZZIN deficiency are remediated by cardiolipin-interacting small molecules
- Diversities in Leigh Syndrome Associated with MT-ATP6 Gene Variants
- Duplexing complexome profiling with SILAC to study human respiratory chain assembly defects
- Early embryonic lethality in complex I associated p.L104P Nubpl mutant mice
- Early Forms of alpha-Synuclein Pathology Are Associated with Neuronal Complex I Deficiency in the Substantia Nigra of Individuals with Parkinson's Disease
- Effects of clofibrate and KH176 on life span and motor function in mitochondrial complex I-deficient mice
- EVT-701 is a novel selective and safe mitochondrial complex 1 inhibitor with potent anti-tumor activity in models of solid cancers
- First-line Screening of OXPHOS Deficiencies Using Microscale Oxygraphy in Human Skin Fibroblasts: A Preliminary Study
- Fut2 Deficiency Promotes Intestinal Stem Cell Aging by Damaging Mitochondrial Functions via Down-Regulating alpha1,2-Fucosylation of Asah2 and Npc1
- Galectin-3 modulates epithelial cell adaptation to stress at the ER-mitochondria interface
- Genetic Reduction of Mitochondria Complex I Subunits is Protective against Cisplatin-Induced Neurotoxicity in Drosophila
- Glucose 6-P dehydrogenase delays the onset of frailty by protecting against muscle damage
- Glutamine depletion disrupts mitochondrial integrity and impairs C2C12 myoblast proliferation, differentiation, and the heat-shock response
- Hypoxia ameliorates brain hyperoxia and NAD(+) deficiency in a murine model of Leigh syndrome
- Hypoxia Promotes Mitochondrial Complex I Abundance via HIF-1alpha in Complex III and Complex IV Eficient Cells
- Identification of two novel variants of BCS1L gene in a patient with classical GRACILE syndrome
- Impairment of Mitochondrial Redox Status in Peripheral Lymphocytes of Multiple Sclerosis Patients
- In vivo magnetic resonance spectroscopy in the brain of Cdkl5 null mice reveals a metabolic profile indicative of mitochondrial dysfunctions
- Increased reactive oxygen species production and maintenance of membrane potential in VDAC-less Neurospora crassa mitochondria
- Inhibition of pyrimidine biosynthesis by strobilurin derivatives induces differentiation of acute myeloid leukemia cells
- Inhibition of receptor-interacting protein kinase 1 improves experimental non-alcoholic fatty liver disease
- IQGAP1 mediates the communication between the nucleus and the mitochondria via NDUFS4 alternative splicing
- Iron-sulfur cluster ISD11 deficiency (LYRM4 gene) presenting as cardiorespiratory arrest and 3-methylglutaconic aciduria
- ISCA2 deficiency leads to heme synthesis defects and impaired erythroid differentiation in K562 cells by indirect ROS-mediated IRP1 activation
- Lack of mitochondrial complex I assembly factor NDUFAF2 results in a distinctive infantile-onset brainstem neurodegenerative disease with early lethality
- Long-term prognosis and genetic background of cardiomyopathy in 223 pediatric mitochondrial disease patients
- Loss of COX4I1 Leads to Combined Respiratory Chain Deficiency and Impaired Mitochondrial Protein Synthesis
- Loss of DIAPH1 causes SCBMS, combined immunodeficiency, and mitochondrial dysfunction
- LYRM7-associated mitochondrial complex III deficiency with non-cavitating leukoencephalopathy and stroke-like episodes
- Mammalian NSUN2 introduces 5-methylcytidines into mitochondrial tRNAs
- Mesenchymal stem cells improve redox homeostasis and mitochondrial respiration in fibroblast cell lines with pathogenic MT-ND3 and MT-ND6 variants
- Metabolic rescue ameliorates mitochondrial encephalo-cardiomyopathy in murine and human iPSC models of Leigh syndrome
- Metformin as a Treatment Strategy for Sjögren's Syndrome
- Metformin delays neurological symptom onset in a mouse model of neuronal complex I deficiency
- Methylglyoxal-mediated Gpd1 activation restores the mitochondrial defects in a yeast model of mitochondrial DNA depletion syndrome
- Mitochondrial dysfunction abrogates dietary lipid processing in enterocytes
- Mitochondrial haplotype mutation alleviates respiratory defect of MELAS by restoring taurine modification in tRNA with 3243A > G mutation
- Mitochondrial respiration supports autophagy to provide stress resistance during quiescence
- Mitochondrial Targeting in an Anti-Austerity Approach Involving Bioactive Metabolites Isolated from the Marine-Derived Fungus Aspergillus sp
- Mitochondrial ubiquinol oxidation is necessary for tumour growth
- NDUFS3 depletion permits complex I maturation and reveals TMEM126A/OPA7 as an assembly factor binding the ND4-module intermediate
- Neuroimaging in Primary Coenzyme-Q(10)-Deficiency Disorders
- Nitric Oxide Modulates Metabolic Remodeling in Inflammatory Macrophages through TCA Cycle Regulation and Itaconate Accumulation
- Noninvasive Ophthalmic Imaging Measures Retinal Degeneration and Vision Deficits in Ndufs4-/- Mouse Model of Mitochondrial Complex I Deficiency
- Nox4 Knockout Does Not Prevent Diaphragm Atrophy, Contractile Dysfunction, or Mitochondrial Maladaptation in the Early Phase Post-Myocardial Infarction in Mice
- Opa1 Overexpression Protects from Early-Onset Mpv17<sup>-/-</sup>-Related Mouse Kidney Disease
- Paroxysmal and non-paroxysmal dystonia in 3 patients with biallelic ECHS1 variants: Expanding the neurological spectrum and therapeutic approaches
- Pathogenic SLIRP variants as a novel cause of autosomal recessive mitochondrial encephalomyopathy with complex I and IV deficiency
- Pathogenic variants in SQOR encoding sulfide:quinone oxidoreductase are a potentially treatable cause of Leigh disease
- Pentatricopeptide repeat protein CNS1 regulates maize mitochondrial complex III assembly and seed development
- Physical Exercise and Mitochondrial Disease: Insights From a Mouse Model
- Plastic response of macrophages to metal ions and nanoparticles in time mimicking metal implant body environment
- Population genetics of Aedes albopictus in the port cities of Hainan Island and Leizhou Peninsula, China
- Promising 8-Aminoquinoline-Based Metal Complexes in the Modulation of SIRT1/3-FOXO3a Axis against Oxidative Damage-Induced Preclinical Neurons
- Protective Effect of Sulforaphane on Oxidative Stress and Mitochondrial Dysfunction Associated with Status Epilepticus in Immature Rats
- Respiratory supercomplexes act as a platform for complex III-mediated maturation of human mitochondrial complexes I and IV
- Scd1 controls de novo beige fat biogenesis through succinate-dependent regulation of mitochondrial complex II
- Selective NADH communication from alpha-ketoglutarate dehydrogenase to mitochondrial transhydrogenase prevents reactive oxygen species formation under reducing conditions in the heart
- Spatiotemporal AMPKalpha2 deletion in mice induces cardiac dysfunction, fibrosis and cardiolipin remodeling associated with mitochondrial dysfunction in males only
- Stress-Induced Mouse Model of the Cardiac Manifestations of Friedreich's Ataxia Corrected by AAV-mediated Gene Therapy
- Successful treatment of infantile-onset ACAD9-related cardiomyopathy with a combination of sodium pyruvate, beta-blocker, and coenzyme Q10
- Sulfite Impairs Bioenergetics and Redox Status in Neonatal Rat Brain: Insights into the Early Neuropathophysiology of Isolated Sulfite Oxidase and Molybdenum Cofactor Deficiencies
- Systematic analysis of a mitochondrial disease-causing ND6 mutation in mitochondrial deficiency
- Telencephalon-specific Alkbh1 conditional knockout mice display hippocampal atrophy and impaired learning
- The Effect of Methylmalonic Acid Treatment on Human Neuronal Cell Coenzyme Q(10) Status and Mitochondrial Function
- The Effect of Organophosphate Exposure on Neuronal Cell Coenzyme Q<sub>10</sub> Status
- The Potential Therapeutic Role of Metformin in Diabetic and Non-Diabetic Bone Impairment
- Total and reduced/oxidized forms of coenzyme Q(10) in fibroblasts of patients with mitochondrial disease
- Two independent respiratory chains adapt OXPHOS performance to glycolytic switch
- Uniparental isodisomy as a cause of mitochondrial complex I respiratory chain disorder due to a novel splicing NDUFS4 mutation
- Vanadium: Risks and possible benefits in the light of a comprehensive overview of its pharmacotoxicological mechanisms and multi-applications with a summary of further research trends
- Yeast Translational Activator Mss51p and Human ZMYND17 - Two Proteins with a Common Origin, but Different Functions