Coenzyme Q10 Supplementation in a Child with Biallelic COQ8A Variants: A Case Report.

Mitochondrial Dysfunctions in Human Primary Coenzyme Q10 Deficiencies.

Intermittent ketogenic fasting with medium-chain triglycerides improves ataxia in COQ8A-related coenzyme Q10 deficiency: A case report.

Case Report: Myoclonic and tremulous movements associated with COQ8A-related coenzyme Q10 deficiency type 4.

Clinical, genetic, and advanced neuroimaging features in adult siblings with Q10 deficiency due to COQ4 mutation: Review of literature.

Ferroptosis susceptibility in primary coenzyme Q10 deficiency: Cellular insights from patient fibroblasts and clinical course of six individuals.

Successful Living Kidney Donation from Heterozygous Carrier Parents to Siblings with Coenzyme Q8B Nephropathy: Two Case Series.

Network Pharmacology Insights Into Statin-Induced Coenzyme Q10 Deficiency: Lipid Metabolic Crosstalk, TNF-MAPK Signaling, and Muscle Toxicity.

Prenatal Diagnosis of COQ2 Variants in Suspected Coenzyme Q10 Deficiency.

Severe Neurological Presentation in Siblings With COQ5-Related Primary Coenzyme Q10 Deficiency: Expanding Clinical and Molecular Spectrum.

Mitochondrial redox imbalance and CoQ10 deficiency in Rett syndrome: Insights from patient-derived fibroblasts.

Fetal Anemia and Periventricular Hyperechogenicity/Halo as a Prenatal Indicator of CoQ10 Deficiency Associated With Biallelic Variant in COQ2 Gene.

CRISPR/Cas9-mediated editing of COQ4 in induced pluripotent stem cells: A model for investigating COQ4-associated human coenzyme Q10 deficiency.

Generation of an induced pluripotent stem cell (iPSC) line (XMUi001-A) derived from a patient harboring homozygous mutations c.370G > A (p.Gly124Ser) in the COQ4 gene.

Coenzyme Q headgroup intermediates can ameliorate a mitochondrial encephalopathy.

Blended Phenotypes of Sexual Development Disorder and Coenzyme Q10 Deficiency, Together with a Sibling with Homozygous Variants in the AHI1 Gene.

A high-dose coenzyme Q10-emulgel for chronic oral therapy of deficient patients with secondary dysphagia.

Tremors with dystonia as a predominant manifestation of primary coenzyme Q10 deficiency.

Two cases of neonatal hyperglycemia caused by a homozygous COQ9 stop-gain variant.

Modelling the human coenzyme Q deficiency in Drosophila melanogaster.

Coenzyme Q10 deficiency disrupts lipid metabolism by altering cholesterol homeostasis in neurons.

Identification of a new COQ4 spliceogenic variant causing severe primary coenzyme Q deficiency.

Syndromic retinitis pigmentosa.

Adult-onset status epilepticus in patients with COQ8A coenzyme Q10 deficiency: A case series.

[Diagnosis and treatment of primary coenzyme Q10 deficiency associated glomerulopathy].

The Spectrum of clinical manifestations in newborns with the COQ4 mutation: case series and literature review.

Familial thrombotic microangiopathy in a child with coenzyme Q10 deficiency-associated glomerulopathy.

New variants expand the neurological phenotype of COQ7 deficiency.

Alterations in coenzyme Q10 status in a cybrid line harboring the 3243A>G mutation of mitochondrial DNA is associated with abnormal mitochondrial bioenergetics and dysregulated mitochondrial biogenesis.

The Ubiquinone-Ubiquinol Redox Cycle and Its Clinical Consequences: An Overview.

The Effect of Neuronal CoQ10 Deficiency and Mitochondrial Dysfunction on a Rotenone-Induced Neuronal Cell Model of Parkinson's Disease.

Coenzyme Q deficiency may predispose to sudden unexplained death via an increased risk of cardiac arrhythmia.

Glomerular basement membrane ultrastructural changes in a patient with COQ2 glomerulopathy: A case report.

Stroke-Like Lesions or Epiphenomena of Seizures in COQ8A -Related Coenzyme-Q10 Deficiency.

COQ7 defect causes prenatal onset of mitochondrial CoQ10 deficiency with cardiomyopathy and gastrointestinal obstruction.

4-Hydroxybenzoic acid rescues multisystemic disease and perinatal lethality in a mouse model of mitochondrial disease.

Primary Coenzyme Q10 Deficiency-Related Ataxias.

Coenzyme Q4 is a functional substitute for coenzyme Q10 and can be targeted to the mitochondria.

Primary Coenzyme Q10 Deficiency-4 Causing Young Onset Ataxia-Dystonia.

Metabolic etiologies in children with infantile epileptic spasm syndrome: Experience at a tertiary pediatric neurology center.

Mitochondrial Dysfunction due to Novel COQ8A Variation with Poor Response to CoQ10 Treatment: A Comprehensive Study and Review of Literatures.

Coenzyme Q deficiency in endothelial mitochondria caused by hypoxia; remodeling of the respiratory chain and sensitivity to anoxia/reoxygenation.

Two Turkish patients with Primary Coenzyme Q10 Deficiency-7: case report and literature review.

Mutations of GEMIN5 are associated with coenzyme Q10 deficiency: long-term follow-up after treatment.

Stroke-Like Episodes and Epilepsy in a Patient with COQ8A-Related Coenzyme Q10 Deficiency.

Brown adipose tissue CoQ deficiency activates the integrated stress response and FGF21-dependent mitohormesis.

Epilepsy and Coenzyme Q10 deficiency with COQ4 variants.

Distal hereditary motor neuropathy caused by coenzyme Q deficiency due to COQ7 variants.

Ataxia due to a COQ8A Novel Variant in Primary Coenzyme Q10 Deficiency.

Primary Coenzyme Q10 Deficiency: An Update.

Adolescence Onset Primary Coenzyme Q10 Deficiency With Rare CoQ8A Gene Mutation: A Case Report and Review of Literature.

Phenotypic, molecular, and functional characterization of COQ7-related primary CoQ10 deficiency: Hypomorphic variants and two distinct disease entities.

Biallelic variants in COQ7 cause distal hereditary motor neuropathy with upper motor neuron signs.

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.

Characterization and bioavailability of a novel coenzyme Q10 nanoemulsion used as an infant formula supplement.

Evaluation of Coenzyme Q10 (CoQ10) Deficiency and Therapy in Mouse Models of Cardiomyopathy.

Total and reduced/oxidized forms of coenzyme Q10 in fibroblasts of patients with mitochondrial disease.

Depletion and Supplementation of Coenzyme Q10 in Secondary Deficiency Disorders.

Homozygous COQ7 mutation: a new cause of potentially treatable distal hereditary motor neuropathy.

Retinopathy and optic atrophy in a case of COQ2-related primary coenzyme Q10 deficiency.

Coenzyme Q10 in aging and disease.

COQ8A-Ataxia as a Manifestation of Primary Coenzyme Q Deficiency.

Manganese-driven CoQ deficiency.

Reduced prosaposin levels in HepG2 cells with long-term coenzyme Q10 deficiency.

Effects of CoQ10 Replacement Therapy on the Audiological Characteristics of Pediatric Patients with COQ6 Variants.

Treatable Ataxias: How to Find the Needle in the Haystack?

Bi-Allelic COQ4 Variants Cause Adult-Onset Ataxia-Spasticity Spectrum Disease.

The efficacy of coenzyme Q10 treatment in alleviating the symptoms of primary coenzyme Q10 deficiency: A systematic review.

Investigation of coenzyme Q10 status, serum amyloid-β, and tau protein in patients with dementia.

Expanding the clinical spectrum of primary coenzyme Q10 deficiency type 6: The first case with cardiomyopathy.

Primary CoQ10 deficiency with a severe phenotype due to the c.901 C > T (p.R301W) mutation in the COQ8A gene.

Oral Coenzyme Q10 supplementation leads to better preservation of kidney function in steroid-resistant nephrotic syndrome due to primary Coenzyme Q10 deficiency.

The cerebellar bioenergetic state predicts treatment response in COQ8A-related ataxia.

Altered brown adipose tissue mitochondrial function in newborn fragile X syndrome mice.

Variation of the clinical spectrum and genotype-phenotype associations in Coenzyme Q10 deficiency associated glomerulopathy.

Coenzyme Q10 deficiency can be expected to compromise Sirt1 activity.

Epilepsia Partialis Continua a Clinical Feature of a Missense Variant in the ADCK3 Gene and Poor Response to Therapy.

Primary Coenzyme Q10 Deficiency-7 and Pathogenic COQ4 Variants: Clinical Presentation, Biochemical Analyses, and Treatment.

A Family Segregating Lethal Primary Coenzyme Q10 Deficiency Due to Two Novel COQ6 Variants.

The Roles of Coenzyme Q in Disease: Direct and Indirect Involvement in Cellular Functions.

Cellular Models for Primary CoQ Deficiency Pathogenesis Study.

Coenzyme Q nanodisks counteract the effect of statins on C2C12 myotubes.

Unraveling the genetic complexities of combined retinal dystrophy and hearing impairment.

Coenzyme Q10 supplementation - In ageing and disease.

Secondary CoQ10 deficiency, bioenergetics unbalance in disease and aging.

Primary Coenzyme Q deficiencies: A literature review and online platform of clinical features to uncover genotype-phenotype correlations.

Coenzyme Q10 deficiency in patients with hereditary hemochromatosis.

The multiple roles of coenzyme Q in cellular homeostasis and their relevance for the pathogenesis of coenzyme Q deficiency.

Photoparoxysmal response in ADCK3 autosomal recessive ataxia: a case report and literature review.

Coenzyme Q10 for Patients With Cardiovascular Disease: JACC Focus Seminar.

Coenzyme Q10 for heart failure.

Secondary coenzyme Q deficiency in neurological disorders.

Targeting a Braf/Mapk pathway rescues podocyte lipid peroxidation in CoQ-deficiency kidney disease.

Redefining infantile-onset multisystem phenotypes of coenzyme Q10-deficiency in the next-generation sequencing era.

Cellular Consequences of Coenzyme Q10 Deficiency in Neurodegeneration of the Retina and Brain.

Missense PDSS1 mutations in CoenzymeQ10 synthesis cause optic atrophy and sensorineural deafness.

Clinical spectrum in multiple families with primary COQ10 deficiency.

Compound heterozygous inheritance of two novel COQ2 variants results in familial coenzyme Q deficiency.

Laboratory Diagnosis of a Case with Coenzyme Q10 Deficiency.

[Primary coenzyme Q10 deficiency-7: a case report and literature review].

CoQ10 Deficient Endothelial Cell Culture Model for the Investigation of CoQ10 Blood-Brain Barrier Transport.

Coenzyme Q10 modulates sulfide metabolism and links the mitochondrial respiratory chain to pathways associated to one carbon metabolism.

Mitochondrial Disease and Coenzyme Q10 Deficiency: Commentary.

Disorders of Human Coenzyme Q10 Metabolism: An Overview.

Coenzyme Q biosynthesis inhibition induces HIF-1α stabilization and metabolic switch toward glycolysis.

Mitochondrial energetic impairment in a patient with late-onset glutaric acidemia Type 2.

Primary coenzyme Q10 deficiency due to COQ8A gene mutations.

Early-onset coenzyme Q10 deficiency associated with ataxia and respiratory chain dysfunction due to novel pathogenic COQ8A variants, including a large intragenic deletion.

Metabolism of the Flavonol Kaempferol in Kidney Cells Liberates the B-ring to Enter Coenzyme Q Biosynthesis.

A novel COQ8A missense variant associated with a mild form of primary coenzyme Q10 deficiency type 4.

Clinical Evidence for Q10 Coenzyme Supplementation in Heart Failure: From Energetics to Functional Improvement.

Coenzyme Q10 Assessment and the Establishment of a Neuronal Cell Model of CoQ10 Deficiency.

Coenzyme Q10 status, glucose parameters, and antioxidative capacity in college athletes.

A rare case of primary coenzyme Q10 deficiency due to COQ9 mutation.

Design of High-Throughput Screening of Natural Extracts to Identify Molecules Bypassing Primary Coenzyme Q Deficiency in Saccharomyces cerevisiae.

A steroid-resistant nephrotic syndrome in an infant resulting from a consanguineous marriage with COQ2 and ARSB gene mutations: a case report.

Dystonia-Ataxia with early handwriting deterioration in COQ8A mutation carriers: A case series and literature review.

The Paradox of Coenzyme Q10 in Aging.

Fetal coenzyme Q10 deficiency in intrahepatic cholestasis of pregnancy.

Primary coenzyme Q10 deficiency-7: expanded phenotypic spectrum and a founder mutation in southern Chinese.

Clinical whole-exome sequencing reveals a common pathogenic variant in patients with CoQ10 deficiency: An underdiagnosed cause of mitochondriopathy.

Human COQ10A and COQ10B are distinct lipid-binding START domain proteins required for coenzyme Q function.

Vitamin K2 cannot substitute Coenzyme Q10 as electron carrier in the mitochondrial respiratory chain of mammalian cells.

Alterations of Mitochondrial Biology in the Oral Mucosa of Chilean Children with Autism Spectrum Disorder (ASD).

Primary Coenzyme Q deficiency Due to Novel ADCK3 Variants, Studies in Fibroblasts and Review of Literature.

Association between Crohn's disease and AarF domain-containing kinase 4 glomerulopathy.

Primary coenzyme Q10 Deficiency-6 (COQ10D6): Two siblings with variable expressivity of the renal phenotype.

Clinical phenotype, in silico and biomedical analyses, and intervention for an East Asian population-specific c.370G>A (p.G124S) COQ4 mutation in a Chinese family with CoQ10 deficiency-associated Leigh syndrome.

ADCK3-related Coenzyme Q10 Deficiency: A Potentially Treatable Genetic Disease.

A study in a Polish ataxia cohort indicates genetic heterogeneity and points to MTCL1 as a novel candidate gene.

Intracellular cholesterol accumulation and coenzyme Q10 deficiency in Familial Hypercholesterolemia.

Increased oxidative stress and coenzyme Q10 deficiency in centenarians.

CoQ10 supplementation rescues nephrotic syndrome through normalization of H2S oxidation pathway.

CoQ10-related sustained remission of proteinuria in a child with COQ6 glomerulopathy-a case report.

Coenzyme Q10 deficiency due to a COQ4 gene defect causes childhood-onset spinocerebellar ataxia and stroke-like episodes.

[Clinical analysis of one infantile nephrotic syndrome caused by COQ2 gene mutation and literature review].

Clinical syndromes associated with Coenzyme Q10 deficiency.

Coenzyme Q10 deficiencies: pathways in yeast and humans.

Laboratory investigations.

Molecular diagnosis of coenzyme Q10 deficiency: an update.

The idebenone metabolite QS10 restores electron transfer in complex I and coenzyme Q defects.

COQ2 nephropathy: a treatable cause of nephrotic syndrome in children.

A family segregating lethal neonatal coenzyme Q10 deficiency caused by mutations in COQ9.

The dilemma of diagnosing coenzyme Q10 deficiency in muscle.

Mitochondrial CoQ deficiency is a common driver of mitochondrial oxidants and insulin resistance.

Estimating the occurrence of primary ubiquinone deficiency by analysis of large-scale sequencing data.

Bypassing human CoQ10 deficiency.

Transcriptomic and proteomic landscape of mitochondrial dysfunction reveals secondary coenzyme Q deficiency in mammals.

Diffuse mesangial sclerosis in a PDSS2 mutation-induced coenzyme Q10 deficiency.

Detection of 6-demethoxyubiquinone in CoQ10 deficiency disorders: Insights into enzyme interactions and identification of potential therapeutics.

Genetic Rescue of Mitochondrial and Skeletal Muscle Impairment in an Induced Pluripotent Stem Cells Model of Coenzyme Q10 Deficiency.

Focal segmental glomerulosclerosis and medullary nephrocalcinosis in children with ADCK4 mutations.

The Value of Coenzyme Q10 Determination in Mitochondrial Patients.

[Primary coenzyme Q10 deficiency type 1 in nephrotic syndrome].

COQ6 Mutations in Children With Steroid-Resistant Focal Segmental Glomerulosclerosis and Sensorineural Hearing Loss.

Further phenotypic heterogeneity of CoQ10 deficiency associated with steroid resistant nephrotic syndrome and novel COQ2 and COQ6 variants.

Statins barely touch the heart but bite the kidneys after cardiac surgery. Coenzyme Q10 deficiency in the dock?

CoQ deficiency causes disruption of mitochondrial sulfide oxidation, a new pathomechanism associated with this syndrome.

Coenzyme Q deficiency causes impairment of the sulfide oxidation pathway.

A Novel SUCLA2 Mutation Presenting as a Complex Childhood Movement Disorder.

The COQ2 genotype predicts the severity of coenzyme Q10 deficiency.

Atypical parkinsonism - new advances.

Decreased Coenzyme Q10 Levels in Multiple System Atrophy Cerebellum.

Disruption of the human COQ5-containing protein complex is associated with diminished coenzyme Q10 levels under two different conditions of mitochondrial energy deficiency.

Genome-wide association study of serum coenzyme Q10 levels identifies susceptibility loci linked to neuronal diseases.

ADCK3 mutations with epilepsy, stroke-like episodes and ataxia: a POLG mimic?

Coenzyme Q10 analytical determination in biological matrices and pharmaceuticals.

Coenzyme Q biosynthesis in health and disease.

Executive and Attentional Disorders, Epilepsy and Porencephalic Cyst in Autosomal Recessive Cerebellar Ataxia Type 3 Due to ANO10 Mutation.

Statin-associated cerebellar ataxia. A Brazilian case series.

Cerebellar ataxia and severe muscle CoQ10 deficiency in a patient with a novel mutation in ADCK3.

Coenzyme Q10 defects may be associated with a deficiency of Q10-independent mitochondrial respiratory chain complexes.

The Interaction Between Statins and Exercise: Mechanisms and Strategies to Counter the Musculoskeletal Side Effects of This Combination Therapy.

MicroRNAs as biomarkers of hepatotoxicity in a randomized placebo-controlled study of simvastatin and ubiquinol supplementation.

NOVEL MUTATION OF THE ELECTRON TRANSFERRING FLAVOPROTEIN DEHYDROGENASE (ETFDH) GENE IN THE ISOLATED MYOPATHIC FORM OF COENZYME q10 DEFICIENCY.

Molecular diagnosis of coenzyme Q10 deficiency.

Rescue of primary ubiquinone deficiency due to a novel COQ7 defect using 2,4-dihydroxybensoic acid.

A novel non-invasive sampling method using buccal mucosa cells for determination of coenzyme Q10.

The clinical heterogeneity of coenzyme Q10 deficiency results from genotypic differences in the Coq9 gene.

COQ4 mutations cause a broad spectrum of mitochondrial disorders associated with CoQ10 deficiency.

Dependence of brown adipose tissue function on CD36-mediated coenzyme Q uptake.

Primary coenzyme Q10 deficiency presenting as fatal neonatal multiorgan failure.