• <em>COQ8A</em>-Ataxia as a Manifestation of Primary Coenzyme Q Deficiency
• 4-Hydroxybenzoic acid rescues multisystemic disease and perinatal lethality in a mouse model of mitochondrial disease
• A case of combined oxidative phosphorylation deficiency 32 caused by MRPS34 gene variation and literature review
• A Family Segregating Lethal Primary Coenzyme Q10 Deficiency Due to Two Novel <em>COQ6</em> Variants
• A Family Segregating Lethal Primary Coenzyme Q10 Deficiency Due to Two Novel COQ6 Variants
• Adolescence Onset Primary Coenzyme Q10 Deficiency With Rare CoQ8A Gene Mutation: A Case Report and Review of Literature
• Altered brown adipose tissue mitochondrial function in newborn fragile X syndrome mice
• Ataxia due to a COQ8A Novel Variant in Primary Coenzyme Q10 Deficiency
• Bi-Allelic COQ4 Variants Cause Adult-Onset Ataxia-Spasticity Spectrum Disease
• Biallelic variants in COQ7 cause distal hereditary motor neuropathy with upper motor neuron signs
• Brown adipose tissue CoQ deficiency activates the integrated stress response and FGF21-dependent mitohormesis
• Cellular Consequences of Coenzyme Q10 Deficiency in Neurodegeneration of the Retina and Brain
• Cellular Models for Primary CoQ Deficiency Pathogenesis Study
• Characterization and bioavailability of a novel coenzyme Q₁₀ nanoemulsion used as an infant formula supplement
• Clinical spectrum in multiple families with primary COQ₁₀ deficiency
• Coenzyme Q biochemistry and biosynthesis
• Coenzyme Q biosynthesis inhibition induces HIF-1α stabilization and metabolic switch toward glycolysis
• Coenzyme Q deficiency in endothelial mitochondria caused by hypoxia; remodeling of the respiratory chain and sensitivity to anoxia/reoxygenation
• Coenzyme Q nanodisks counteract the effect of statins on C2C12 myotubes
• Coenzyme Q₁₀ for Patients With Cardiovascular Disease: JACC Focus Seminar
• Coenzyme Q₁₀ in aging and disease
• Coenzyme Q₁₀ supplementation - In ageing and disease
• Coenzyme Q₄ is a functional substitute for coenzyme Q₁₀ and can be targeted to the mitochondria
• Coenzyme Q10 and Endocrine Disorders: An Overview
• Coenzyme Q10 deficiency can be expected to compromise Sirt1 activity
• Coenzyme Q10 deficiency in patients with hereditary hemochromatosis
• Coenzyme Q10 for enhancing physical activity and extending the human life cycle
• Coenzyme Q10 for heart failure
• Coenzyme Q10 modulates sulfide metabolism and links the mitochondrial respiratory chain to pathways associated to one carbon metabolism
• Compound heterozygous inheritance of two novel COQ2 variants results in familial coenzyme Q deficiency
• Congenital coenzyme Q5-linked pathology: causal genetic association, core phenotype, and molecular mechanism
• CoQ₁₀ Deficient Endothelial Cell Culture Model for the Investigation of CoQ₁₀ Blood-Brain Barrier Transport
• COQ7 defect causes prenatal onset of mitochondrial CoQ₁₀ deficiency with cardiomyopathy and gastrointestinal obstruction
• Curcumin Induces Ferroptosis in A549 CD133⁺ Cells through the GSH-GPX4 and FSP1-CoQ10-NAPH Pathways
• Depletion and Supplementation of Coenzyme Q10 in Secondary Deficiency Disorders
• Disorders of Human Coenzyme Q10 Metabolism: An Overview
• Distal hereditary motor neuropathy caused by coenzyme Q deficiency due to COQ7 variants
• Effect of coenzyme Q10 on cardiac function and survival in heart failure: an overview of systematic reviews and <em>meta</em>-analyses
• Effects of arginine on coenzyme-Q10 micelle uptake for mitochondria-targeted nanotherapy in phenylketonuria
• Effects of CoQ10 Replacement Therapy on the Audiological Characteristics of Pediatric Patients with <em>COQ6</em> Variants
• Effects of Ndufs4 Deletion on Hearing after Various Acoustic Exposures
• Epilepsia Partialis Continua a Clinical Feature of a Missense Variant in the ADCK3 Gene and Poor Response to Therapy
• Epilepsy and Coenzyme Q10 deficiency with COQ4 variants
• Evaluation of Coenzyme Q10 (CoQ10) Deficiency and Therapy in Mouse Models of Cardiomyopathy
• Expanding the clinical spectrum of primary coenzyme Q10 deficiency type 6: The first case with cardiomyopathy
• Glomerular basement membrane ultrastructural changes in a patient with COQ2 glomerulopathy: A case report
• Homozygous COQ7 mutation: a new cause of potentially treatable distal hereditary motor neuropathy
• Investigation of coenzyme Q10 status, serum amyloid-β, and tau protein in patients with dementia
• Iron status influences mitochondrial disease progression in Complex I-deficient mice
• Laboratory Diagnosis of a Case with Coenzyme Q10 Deficiency
• Manganese-driven CoQ deficiency
• Metabolic etiologies in children with infantile epileptic spasm syndrome: Experience at a tertiary pediatric neurology center
• Microbial rhodoquinone biosynthesis proceeds via an atypical RquA-catalyzed amino transfer from S-adenosyl-L-methionine to ubiquinone
• Micronutrients/miRs/ATP networking in mitochondria: Clinical intervention with ferroptosis, cuproptosis, and calcium burden
• Missense PDSS1 mutations in CoenzymeQ10 synthesis cause optic atrophy and sensorineural deafness
• Mitochondrial Disease and Coenzyme Q10 Deficiency: Commentary
• Mitochondrial energetic impairment in a patient with late-onset glutaric acidemia Type 2
• Mutations of GEMIN5 are associated with coenzyme Q₁₀ deficiency: long-term follow-up after treatment
• NDUFC2 deficiency exacerbates endothelial mesenchymal transformation during ischemia-reperfusion via NLRP3
• Neuroimaging in Primary Coenzyme-Q₁₀-Deficiency Disorders
• New Insights on the Uptake and Trafficking of Coenzyme Q
• Novel Homozygous Variant in <em>COQ7</em> in Siblings With Hereditary Motor Neuropathy
• Oral Coenzyme Q10 supplementation leads to better preservation of kidney function in steroid-resistant nephrotic syndrome due to primary Coenzyme Q10 deficiency
• PHB2 ameliorates Doxorubicin-induced cardiomyopathy through interaction with NDUFV2 and restoration of mitochondrial complex I function
• Phenotypic, molecular, and functional characterization of COQ7-related primary CoQ₁₀ deficiency: Hypomorphic variants and two distinct disease entities
• Photoparoxysmal response in ADCK3 autosomal recessive ataxia: a case report and literature review
• Primary Coenzyme Q deficiencies: A literature review and online platform of clinical features to uncover genotype-phenotype correlations
• Primary Coenzyme Q10 Deficiency-4 Causing Young Onset Ataxia-Dystonia
• Primary Coenzyme Q10 Deficiency-7 and Pathogenic <em>COQ4</em> Variants: Clinical Presentation, Biochemical Analyses, and Treatment
• Primary Coenzyme Q10 Deficiency-7 and Pathogenic COQ4 Variants: Clinical Presentation, Biochemical Analyses, and Treatment
• Primary coenzyme Q10 deficiency-7: a case report and literature review
• Primary Coenzyme Q10 Deficiency-Related Ataxias
• Primary Coenzyme Q10 Deficiency: An Update
• Primary CoQ₁₀ deficiency with a severe phenotype due to the c.901 C &gt; T (p.R301W) mutation in the COQ8A gene
• Redefining infantile-onset multisystem phenotypes of coenzyme Q₁₀-deficiency in the next-generation sequencing era
• Reduced prosaposin levels in HepG2 cells with long-term coenzyme Q10 deficiency
• Retinopathy and optic atrophy in a case of <em>COQ2</em>-related primary coenzyme Q₁₀ deficiency
• Secondary coenzyme Q deficiency in neurological disorders
• Secondary CoQ₁₀ deficiency, bioenergetics unbalance in disease and aging
• Stroke-Like Episodes and Epilepsy in a Patient with COQ8A-Related Coenzyme Q10 Deficiency
• Stroke-Like Lesions or Epiphenomena of Seizures in COQ8A-Related Coenzyme-Q10 Deficiency
• Structure of the human respiratory complex II
• Targeting a Braf/Mapk pathway rescues podocyte lipid peroxidation in CoQ-deficiency kidney disease
• The cerebellar bioenergetic state predicts treatment response in COQ8A-related ataxia
• The Effect of Neuronal CoQ₁₀ Deficiency and Mitochondrial Dysfunction on a Rotenone-Induced Neuronal Cell Model of Parkinson's Disease
• The efficacy of coenzyme Q₁₀ treatment in alleviating the symptoms of primary coenzyme Q₁₀ deficiency: A systematic review
• The Immunoprotection of <em>OmpH</em> Gene Deletion Mutation of <em>Pasteurella multocida</em> on Hemorrhagic Sepsis in Qinghai Yak
• The multiple roles of coenzyme Q in cellular homeostasis and their relevance for the pathogenesis of coenzyme Q deficiency
• The Rationale for Vitamin, Mineral, and Cofactor Treatment in the Precision Medical Care of Autism Spectrum Disorder
• The Roles of Coenzyme Q in Disease: Direct and Indirect Involvement in Cellular Functions
• The Ubiquinone-Ubiquinol Redox Cycle and Its Clinical Consequences: An Overview
• Total and reduced/oxidized forms of coenzyme Q₁₀ in fibroblasts of patients with mitochondrial disease
• Treatable Ataxias: How to Find the Needle in the Haystack?
• Two Patients Diagnosed as Succinate Dehydrogenase Deficiency: Case Report
• Two Turkish patients with Primary Coenzyme Q10 Deficiency-7: case report and literature review
• Ubiquinone deficiency drives reverse electron transport to disrupt hepatic metabolic homeostasis in obesity
• Understanding the Pathogenesis of Cardiac Complications in Patients with Propionic Acidemia and Exploring Therapeutic Alternatives for Those Who Are Not Eligible or Are Waiting for Liver Transplantation
• Unraveling the genetic complexities of combined retinal dystrophy and hearing impairment
• Variation of the clinical spectrum and genotype-phenotype associations in Coenzyme Q10 deficiency associated glomerulopathy
• Whole-Exome Sequencing Study of Fibroblasts Derived From Patients With Cerebellar Ataxia Referred to Investigate CoQ10 Deficiency