Hyperoside impairs mitochondrial respiration in chronic myeloid leukemia by promoting STUB1-mediated ubiquitination and degradation of NOX4.

TAZ (Wwtr1) deficiency leads to ER stress and mitochondrial dysfunction in a mouse model of Fuchs' endothelial corneal dystrophy.

A novel mechanism of chlorogenic acid against type 2 diabetes-induced diabetic retinopathy: suppressing ferroptosis via NRF2/xCT/GPX4 and STAT3 signaling.

Colonic spatial single-cell proteomics and murine models link mitochondrial dysfunction to dimeric IgA-secreting plasma cell deficiency in Crohn's disease.

Optic neuropathy arising from the synergy between YARS2 and mitochondrial COX1 mutations.

The metabolic landscape of ovarian cancer stem cells: how do they survive?

Developmental and metabolic toxicity of diphenyl phosphate: insights from an integrative mechanistic framework in zebrafish embryos.

Local mitochondrial physiology defined by mtDNA quality guides purifying selection.

Characterizing the role of mitochondrial dynamics during Drosophila convergent extension using NADH fluorescence lifetime imaging.

Integrated multi-omics profiling of wooden breast reveals energy metabolism dysfunction and RHOA/ROCK pathway activation.

Osteoimmunometabolic modulation via hydrogen-self-supplying magnesium-reinforced collagen membrane for enhanced guided bone regeneration.

Combined ketone body and glutamine supplementation restores aerobic energy production in AGC1-deficient neuronal progenitors.

Tumor-agnostic therapy: a potential therapeutic approach for SMARCA4-deficient malignancies.

Exploring the Protective Effects of Two Alkaloids 1 and 2 from Aspergillus terreus C23-3 on Neuronal Cells by Combining Bioinformatics Prediction and Experimental Verification.

β-Nicotinamide mononucleotide activates the SIRT1/NF-κB pathway to alleviate ischemia reperfusion injury after lung transplantation.

An inherited mitochondrial DNA mutation remodels inflammatory cytokine responses in macrophages and in vivo in mice.

A case of POLG-related mitochondrial DNA maintenance defect.

Gelatin/Graphene Oxide Cryogel as an Artificial Cartilage Substitute Regulates Chondrogenesis and Oxidative Phosphorylation for Osteoarthritis Treatment.

Clinical utility of the ATP hydrolysis assay for the diagnosis of complex V deficiency in cultured skin fibroblasts.

The functional study of novel KLHL3 missense mutations associated with pseudohypoaldosteronism type II.

[Venetoclax resistance and molecular abnormalities in AML].

Exploring the reproductive toxicity and mechanism analysis of perfluorooctanoic acid and perfluorononanoic acid based on network toxicology, molecular docking, and experimental validation.

Gene therapy and mRNA drugs approach for mitochondrial OXPHOS deficiencies.

Defects in anaplerotic metabolism sensitize Staphylococcus aureus small colony variants to bicarbonate.

Altered Heme and Redox Homeostasis Underpin Late-onset Alzheimer's Disease.

Assessing a Mitochondrial Disease Treatment via a Novel Statistical Technique for Accelerometer Data.

Combined effects of nanoplastics and 3-BHA at environmentally relevant concentrations significantly aggravated kidney injury via TGF-β/SMAD signaling pathway in mice.

Antioxidant functionalized double-net/TA dynamic hydrogel promotes cartilage regeneration through stabilization of chondrocyte phenotype.

Non-invasive visualisation of long-lasting brain metabolic alterations in murine pseudo-infection model using parahydrogen-polarised [1-13C] pyruvate MRI.

Integrating multi-omics and machine learning strategies to explore the "gene-protein-metabolite" network in ischemic heart failure with Qi deficiency and blood stasis syndrome.

Bupi Yishen formula improves chronic kidney disease by restoring renal energy metabolism and mitochondrial oxidative phosphorylation.

Microgliosis, neuronal death, minor behavioral abnormalities and reduced endurance performance in alpha-ketoglutarate dehydrogenase complex deficient mice.

OXA1L deficiency causes mitochondrial myopathy via reactive oxygen species regulated nuclear factor kappa B signalling pathway.

Targeting the Electron Transport System for Enhanced Longevity.

Mitochondrial fission factor drives an actionable metabolic vulnerability in multiple myeloma.

Genome sequencing identifies RMND1 as a strong candidate gene for severe prenatal kidney failure mimicking renal tubular dysgenesis associated with hyporeninism.

Further delineation of defects in MRPS2 causing human OXPHOS deficiency and early developmental abnormalities in zebrafish.

Complex Metabolomic Changes in a Combined Defect of Glycosylation and Oxidative Phosphorylation in a Patient with Pathogenic Variants in PGM1 and NDUFA13.

Identifying Aberrant 1CM-Related Pathways by Multi-Omics Analysis and Validating Tumor Inhibitory Effect of One-Carbon Donor Betaine in Gastric Cancer.

Impairment of Muscle Function Causes Pupal Lethality in Flies Expressing the Mitochondrial Alternative Oxidase.

Uncovering Mitochondrial Defects Induced by Chemicals: A Case Study of Low-Dose Medium-Chain Chlorinated Paraffin Exposure.

Targeting STING and protecting mitochondrial function with Nephropathy Ⅱ decoction to alleviate renal fibrosis.

Involvement of Oxidative Stress and Antioxidants in Modification of Cardiac Dysfunction Due to Ischemia-Reperfusion Injury.

Atf3 controls transitioning in female mitochondrial cardiomyopathy as identified by spatial and single-cell transcriptomics.

Comparative Study of Structural and Functional Rearrangements in Skeletal Muscle Mitochondria of SOD1-G93A Transgenic Mice at Pre-, Early-, and Late-Symptomatic Stages of ALS Progression.

Endogenous dual-responsive and self-adaptive silk fibroin-based scaffold with enhancement of immunomodulation for skull regeneration.

A novel polyribonucleotide nucleotidyltransferase 1 (PNPT1) gene variant potentially associated with combined oxidative phosphorylation deficiency 13: case report and literature review.

Divalent metal ions enhance bone regeneration through modulation of nervous systems and metabolic pathways.

Clinical and Genetic Insights Into Combined Oxidative Phosphorylation Defect Type 38.

Mitochondrial abnormalities as a target of intervention in acute myeloid leukemia.

Peroxiredoxin 6 maintains mitochondrial homeostasis and promotes tumor progression through ROS/JNK/p38 MAPK signaling pathway in multiple myeloma.

Multisystem clinicopathologic and genetic analysis of MELAS.

Skeletal muscle proteome differs between young APOE3 and APOE4 targeted replacement mice in a sex-dependent manner.

RMND1 Mutation Case Report and Literature Review.

Intermittent episodes of acute severe encephalomyopathy and early death in two siblings caused by biallelic likely pathogenic variants in FASTKD2: Expanding phenotype and literature review.

Fusogenic Liposomes for the Intracellular Delivery of Phosphocreatine.

Integrative study of skeletal muscle mitochondrial dysfunction in a murine pancreatic cancer-induced cachexia model.

[Mitochondrial metabolism in AML cells].

Biallelic PTPMT1 variants disrupt cardiolipin metabolism and lead to a neurodevelopmental syndrome.

SCUBE3 promotes osteogenic differentiation and mitophagy in human bone marrow mesenchymal stem cells through the BMP2/TGF-β signaling pathway.

Decoding the muscle transcriptome of patients with late-onset Pompe disease reveals markers of disease progression.

Matrix Protein of Vesicular Stomatitis Virus Targets the Mitochondria, Reprograms Glucose Metabolism, and Sensitizes to 2-Deoxyglucose in Glioblastoma.

A roadmap for ribosome assembly in human mitochondria.

Efficacy of Trametinib in Alleviating Cisplatin-Induced Acute Kidney Injury: Inhibition of Inflammation, Oxidative Stress, and Tubular Cell Death in a Mouse Model.

Relationship between Cellular Oxygen Consumption and Atherosclerosis-Associated Mitochondrial Mutations (Variants of the Mitochondrial Genome).

Functional diversity among cardiolipin binding sites on the mitochondrial ADP/ATP carrier.

Reduced Protein Import via TIM23 SORT Drives Disease Pathology in TIMM50-Associated Mitochondrial Disease.

A zebrafish tufm mutant model for the COXPD4 syndrome of aberrant mitochondrial function.

Modulation of Sirtuin 3 by N-Acetylcysteine Preserves Mitochondrial Oxidative Phosphorylation and Restores Bisphenol A-Induced Kidney Damage in High-Fat-Diet-Fed Rats.

Evaluating the toxicity of estetrol, 17α-ethinylestradiol, and their combination with drospirenone on zebrafish larvae: A behavioural and proteomic study.

BmATAD3A mediates mitochondrial ribosomal protein expression to maintain the mitochondrial energy metabolism of the silkworm, Bombyx mori.

Single cell and bulk RNA expression analyses identify enhanced hexosamine biosynthetic pathway and O-GlcNAcylation in acute myeloid leukemia blasts and stem cells.

Expanding the Phenotypic Spectrum: Chronic Kidney Disease in a Patient with Combined Oxidative Phosphorylation Defect 21.

Integrating serum pharmacochemistry and network pharmacology to explore the molecular mechanisms of Acanthopanax senticosus (Rupr. & Maxim.) Harms on attenuating doxorubicin-induced myocardial injury.

Bortezomib in Combination with Physachenolide C Reduces the Tumorigenic Properties of KRASmut/P53mut Lung Cancer Cells by Inhibiting c-FLIP.

Expanding the genetic spectrum of mitochondrial diseases in Tunisia: novel variants revealed by whole-exome sequencing.

A Novel Homozygous Germline Mutation in Transferrin Receptor 1 (TfR1) Leads to Combined Immunodeficiency and Provides New Insights into Iron-Immunity Axis.

Association between glaucoma susceptibility with combined defects in mitochondrial oxidative phosphorylation and fatty acid beta oxidation.

The first case of combined oxidative phosphorylation deficiency-1 due to a GFM1 mutation in the Serbian population: a case report and literature review.

Challenges in Genetic Diagnosis of Mitochondrial Diseases: What Can Functional Genomics' Studies Do?

Functional Diversity of Mammalian Small Heat Shock Proteins: A Review.

Huperzine A injection ameliorates motor and cognitive abnormalities via regulating multiple pathways in a murine model of Parkinson's disease.

Mitochondrial impairment but not peripheral inflammation predicts greater Gulf War illness severity.

TUFM variants lead to white matter abnormalities mimicking multiple sclerosis.

[A case of combined oxidative phosphorylation deficiency 32 caused by MRPS34 gene variation and literature review].

5-Aminolevulinic acid bypasses mitochondrial complex I deficiency and corrects physiological dysfunctions in Drosophila.

A novel biallelic frameshift variant in C2orf69 causing developmental regression, seizures, microcephaly, autistic features, and hypertonia.

SESN2-Mediated AKT/GSK-3β/NRF2 Activation to Ameliorate Adriamycin Cardiotoxicity in High-Fat Diet-Induced Obese Mice.

Severe neonatal onset neuroregression with paroxysmal dystonia and apnoea: Expanding the phenotypic and genotypic spectrum of CARS2-related mitochondrial disease.

Nono deficiency impedes the proliferation and adhesion of H9c2 cardiomyocytes through Pi3k/Akt signaling pathway.

Pathogenicity Analysis of a Novel Variant in GTPBP3 Causing Mitochondrial Disease and Systematic Literature Review.

Anti-AQP4-IgG-positive Leigh syndrome: A case report and review of the literature.

Neuroimaging in mitochondrial disease.

Visualization of mtDNA Using FISH.

Dysregulation of Mitochondrial Translation Caused by CBFB Deficiency Cooperates with Mutant PIK3CA and Is a Vulnerability in Breast Cancer.

Clinical and genetic analyses of premature mitochondrial encephalopathy with epilepsia partialis continua caused by novel biallelic NARS2 mutations.

Predictive plasma biomarker for gestational diabetes: A case-control study in China.

MitoQ alleviates carbon tetrachloride-induced liver fibrosis in mice through regulating JNK/YAP pathway.

Stimulating Mitochondrial Biogenesis with Deoxyribonucleosides Increases Functional Capacity in ECHS1-Deficient Cells.

VARS2 gene mutation leading to overall developmental delay in a child with epilepsy: A case report.

TARS2 Variants Cause Combination Oxidative Phosphorylation Deficiency-21: A Case Report and Literature Review.

A unique insight for Xiaoyao San exerts antidepressant effects by modulating hippocampal glucose catabolism using stable isotope-resolved metabolomics.

Exosome-shuttled mitochondrial transcription factor A mRNA promotes the osteogenesis of dental pulp stem cells through mitochondrial oxidative phosphorylation activation.

Ferroptosis-related gene signature predicts the clinical outcome in pediatric acute myeloid leukemia patients and refines the 2017 ELN classification system.

Functional Restoration of Exhausted CD8 T Cells in Chronic HIV-1 Infection by Targeting Mitochondrial Dysfunction.

Analysis of Organization and Activity of Mitochondrial Respiratory Chain Complexes in Primary Fibroblasts Using Blue Native PAGE.

Whole exome sequencing identifies a novel compound heterozygous GFM1 variant underlying developmental delay, dystonia, polymicrogyria, and severe intellectual disability in a Pakhtun family.

MG53 protein rejuvenates hUC-MSCs and facilitates their therapeutic effects in AD mice by activating Nrf2 signaling pathway.

A Case of Combined Oxidative Phosphorylation Deficiency 35 Associated with a Novel Missense Variant of the TRIT1 Gene.

CRISPR-Cas9 screen identifies oxidative phosphorylation as essential for cancer cell survival at low extracellular pH.

HRAS germline mutations impair LKB1/AMPK signaling and mitochondrial homeostasis in Costello syndrome models.

Novel heterozygous compound TRMT5 mutations associated with combined oxidative phosphorylation deficiency 26 in a Chinese family: a case report.

Combined Treatment with Herbal Medicine and Drug Ameliorates Inflammation and Metabolic Abnormalities in the Liver of an Amyotrophic Lateral Sclerosis Mouse Model.

Clinical and genetic analysis of combined oxidative phosphorylation defificiency-10 caused by MTO1 mutation.

Genetic diagnosis of basal ganglia disease in childhood.

Biological significance of MYC and CEBPD coamplification in urothelial carcinoma: Multilayered genomic, transcriptional and posttranscriptional positive feedback loops enhance oncogenic glycolysis.

High-intensity training induces non-stoichiometric changes in the mitochondrial proteome of human skeletal muscle without reorganisation of respiratory chain content.

The FASTK family proteins fine-tune mitochondrial RNA processing.

Expanding the electro-clinical phenotype of CARS2associated neuroregression.

Curcumin and Carnosic Acid Cooperate to Inhibit Proliferation and Alter Mitochondrial Function of Metastatic Prostate Cancer Cells.

Development and characterization of a mouse model for Acad9 deficiency.

Novel Mutations in the GTPBP3 Gene for Mitochondrial Disease and Characteristics of Related Phenotypic Spectrum: The First Three Cases From China.

APOE genotype dependent molecular abnormalities in the cerebrovasculature of Alzheimer's disease and age-matched non-demented brains.

Pathogenic variants in MRPL44 cause infantile cardiomyopathy due to a mitochondrial translation defect.

COXPD9 in an individual from Puerto Rico and literature review.

Epigenetic changes related to glucose metabolism in type 1 diabetes after BCG vaccinations: A vital role for KDM2B.

Development of cerebral mitochondrial respiratory function is impaired by thyroid hormone deficiency before birth in a region-specific manner.

Mitochondrial metabolism as a target for acute myeloid leukemia treatment.

Metabolic impact of pathogenic variants in the mitochondrial glutamyl-tRNA synthetase EARS2.

Targeting AXL kinase sensitizes leukemic stem and progenitor cells to venetoclax treatment in acute myeloid leukemia.

The role of mitophagy in the regulation of mitochondrial energetic status in neurons.

Uniparental isodisomy of chromosome 2 causing MRPL44-related multisystem mitochondrial disease.

Energy, Entropy and Quantum Tunneling of Protons and Electrons in Brain Mitochondria: Relation to Mitochondrial Impairment in Aging-Related Human Brain Diseases and Therapeutic Measures.

Adenylate kinase 2 expression and addiction in T-ALL.

Dysregulation of Metabolic Pathways in Circulating Natural Killer Cells Isolated from Inflammatory Bowel Disease Patients.

A novel acceptor stem variant in mitochondrial tRNATyr impairs mitochondrial translation and is associated with a severe phenotype.

[Analysis of GFM1 gene mutations in a family with combined oxidative phosphorylation deficiency 1].

Yeast Ppz1 protein phosphatase toxicity involves the alteration of multiple cellular targets.

The roles of SDHAF2 and dicarboxylate in covalent flavinylation of SDHA, the human complex II flavoprotein.

Bioenergetic defects in muscle fibers of RYR1 mutant knock-in mice associated with malignant hyperthermia.

Results of an explorative clinical evaluation suggest immediate and persistent post-reperfusion metabolic paralysis drives kidney ischemia reperfusion injury.

Mitochondria transfer enhances proliferation, migration, and osteogenic differentiation of bone marrow mesenchymal stem cell and promotes bone defect healing.

Protein kinase C-δ interacts with and phosphorylates ARD1.

Biotin, coenzyme Q10, and their combination ameliorate aluminium chloride-induced Alzheimer's disease via attenuating neuroinflammation and improving brain insulin signaling.

TMEM70 functions in the assembly of complexes I and V.

The contribution of ketone bodies to glycolytic inhibition for the treatment of adult and pediatric glioblastoma.

Phenotypes and genotypes of mitochondrial aminoacyl-tRNA synthetase deficiencies from a single neurometabolic clinic.

Metabolic reprogramming of fibro/adipogenic progenitors facilitates muscle regeneration.

Hypomorphic variants in AK2 reveal the contribution of mitochondrial function to B-cell activation.

Inhibition of cAMP/PKA Pathway Protects Optic Nerve Head Astrocytes against Oxidative Stress by Akt/Bax Phosphorylation-Mediated Mfn1/2 Oligomerization.

Clinical Spectrum and Functional Consequences Associated with Bi-Allelic Pathogenic PNPT1 Variants.

Whole-blood transcriptomic responses to lumacaftor/ivacaftor therapy in cystic fibrosis.

[Mitochondrial DNA deletion syndrome: a case report and literature review].

Clinical presentation and proteomic signature of patients with TANGO2 mutations.

Novel alanyl-tRNA synthetase 2 (AARS2) homozygous mutation in a consanguineous Chinese family with premature ovarian insufficiency.

Inhibition of hepatocellular carcinoma by metabolic normalization.

Alterations of skeletal muscle bioenergetics in a mouse with F508del mutation leading to a cystic fibrosis-like condition.

Exophthalmos in Kearns-Sayre syndrome.

A novel de novo MTOR gain-of-function variant in a patient with Smith-Kingsmore syndrome and Antiphospholipid syndrome.

L-Cysteine supplementation prevents liver transplantation in a patient with TRMU deficiency.

Mitochondrial ribosomal protein PTCD3 mutations cause oxidative phosphorylation defects with Leigh syndrome.

A predictive algorithm to identify genes that discriminate individuals with fibromyalgia syndrome diagnosis from healthy controls.

The Role of Sodium in Diabetic Cardiomyopathy.

Early-onset Parkinson disease caused by a mutation in CHCHD2 and mitochondrial dysfunction.

Pathogenic variants in glutamyl-tRNAGln amidotransferase subunits cause a lethal mitochondrial cardiomyopathy disorder.

OXA1L mutations cause mitochondrial encephalopathy and a combined oxidative phosphorylation defect.

Clonal expansion of mtDNA deletions: different disease models assessed by digital droplet PCR in single muscle cells.

Radio-sensitizing effects of VE-821 and beyond: Distinct phosphoproteomic and metabolomic changes after ATR inhibition in irradiated MOLT-4 cells.

Mitochondrial DNA transcription and translation: clinical syndromes.

Combined and individual strategy of exercise generated preconditioning and low dose copper nanoparticles serve as superlative approach to ameliorate ISO-induced myocardial infarction in rats.

Pro-inflammatory cytokines attenuate glucose-stimulated insulin secretion from INS-1E insulinoma cells by restricting mitochondrial pyruvate oxidation capacity - Novel mechanistic insight from real-time analysis of oxidative phosphorylation.

Exercise preconditioning and low dose copper nanoparticles exhibits cardioprotection through targeting GSK-3β phosphorylation in ischemia/reperfusion induced myocardial infarction.

The pathomechanism of cytochrome c oxidase deficiency includes nuclear DNA damage.

High-Resolution FluoRespirometry and OXPHOS Protocols for Human Cells, Permeabilized Fibers from Small Biopsies of Muscle, and Isolated Mitochondria.

Vanishing white matter: a leukodystrophy due to astrocytic dysfunction.

Intratumoral heterogeneity of oxygen metabolism and neovascularization uncovers 2 survival-relevant subgroups of IDH1 wild-type glioblastoma.

Case report: lactic acidosis and rhabdomyolysis during telbivudine and tenofovir treatment for chronic hepatitis B.

Brain lactate and pH in schizophrenia and bipolar disorder: a systematic review of findings from magnetic resonance studies.

Bi-allelic Mutations in the Mitochondrial Ribosomal Protein MRPS2 Cause Sensorineural Hearing Loss, Hypoglycemia, and Multiple OXPHOS Complex Deficiencies.

Mitochondrial Encephalopathy: First Portuguese Report of a VARS2 Causative Variant.

The accumulation of assembly intermediates of the mitochondrial complex I matrix arm is reduced by limiting glucose uptake in a neuronal-like model of MELAS syndrome.

Peculiar combinations of individually non-pathogenic missense mitochondrial DNA variants cause low penetrance Leber's hereditary optic neuropathy.

Building and decoding ubiquitin chains for mitophagy.

The genotypic and phenotypic spectrum of MTO1 deficiency.

Impaired autophagy bridges lysosomal storage disease and epithelial dysfunction in the kidney.

Clinical, biochemical, and genetic features associated with VARS2-related mitochondrial disease.

Pathological mechanisms underlying single large-scale mitochondrial DNA deletions.

Impaired mitochondrial respiration in human carotid plaque atherosclerosis: A potential role for Pink1 in vascular smooth muscle cell energetics.

Melanoma tumors exhibit a variable but distinct metabolic signature.

Functional analysis of Toxoplasma lactate dehydrogenases suggests critical roles of lactate fermentation for parasite growth in vivo.

Prevention of doxorubicin-induced cardiomyopathy using targeted MaFGF mediated by nanoparticles combined with ultrasound-targeted MB destruction.

Lactate-mediated mitoribosomal defects impair mitochondrial oxidative phosphorylation and promote hepatoma cell invasiveness.

Biallelic C1QBP Mutations Cause Severe Neonatal-, Childhood-, or Later-Onset Cardiomyopathy Associated with Combined Respiratory-Chain Deficiencies.

Molecular and clinical spectra of FBXL4 deficiency.

Tigecycline-induced inhibition of mitochondrial DNA translation may cause lethal mitochondrial dysfunction in humans.

Disrupting mitochondrial Ca2+ homeostasis causes tumor-selective TRAIL sensitization through mitochondrial network abnormalities.

Retinopathy and optic atrophy: Expanding the phenotypic spectrum of pathogenic variants in the AARS2 gene.

Impact of mutations within the [Fe-S] cluster or the lipoic acid biosynthesis pathways on mitochondrial protein expression profiles in fibroblasts from patients.

Biallelic Mutations in MRPS34 Lead to Instability of the Small Mitoribosomal Subunit and Leigh Syndrome.