Treatment of CoQ(10) deficient fibroblasts with ubiquinone, CoQ analogs, and vitamin C: time- and compound-dependent effects - Test2 - elhamkhani - TriplyDB

Treatment of CoQ(10) deficient fibroblasts with ubiquinone, CoQ analogs, and vitamin C: time- and compound-dependent effects

Treatment of CoQ(10) deficient fibroblasts with ubiquinone, CoQ analogs, and vitamin C: time- and compound-dependent effects

http://www.wikidata.org/entity/Q28474996

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Idebenone and neuroprotection: antioxidant, pro-oxidant, or electron carrier?The COQ2 genotype predicts the severity of coenzyme Q10 deficiency A water soluble CoQ10 formulation improves intracellular distribution and promotes mitochondrial respiration in cultured cells Dysfunctional Coq9 protein causes predominant encephalomyopathy associated with CoQ deficiency NQO1-dependent redox cycling of idebenone: effects on cellular redox potential and energy levels Mitochondrial dysfunction in inherited renal disease and acute kidney injury Coenzyme Q10 Supplementation and Oocyte Aneuploidy in Women Undergoing IVF-ICSI Treatment.Pathomechanisms in coenzyme q10-deficient human fibroblasts.Effects of inhibiting CoQ10 biosynthesis with 4-nitrobenzoate in human fibroblasts.Analysis of COQ2 gene in multiple system atrophy.Renal mitochondrial cytopathies.176th ENMC International Workshop: diagnosis and treatment of coenzyme Q₁₀ deficiency Renal involvement in mitochondrial cytopathies.The clinical heterogeneity of coenzyme Q10 deficiency results from genotypic differences in the Coq9 gene.CoQ(10) deficiencies and MNGIE: two treatable mitochondrial disorders.Gene Therapy Corrects Mitochondrial Dysfunction in Hematopoietic Progenitor Cells and Fibroblasts from Coq9R239X Mice.The submitochondrial distribution of ubiquinone affects respiration in long-lived Mclk1+/- mice Melatonin enhances neural stem cell differentiation and engraftment by increasing mitochondrial function.Tissue-specific oxidative stress and loss of mitochondria in CoQ-deficient Pdss2 mutant mice.Discovering the link between nutrition and skin aging.Survival transcriptome in the coenzyme Q10 deficiency syndrome is acquired by epigenetic modifications: a modelling study for human coenzyme Q10 deficiencies How mitochondrial dysfunction affects zebrafish development and cardiovascular function: an in vivo model for testing mitochondria-targeted drugs.Coenzyme Q deficiency causes impairment of the sulfide oxidation pathway.CoQ deficiency causes disruption of mitochondrial sulfide oxidation, a new pathomechanism associated with this syndrome.Coenzyme Q and mitochondrial disease Primary and secondary CoQ(10) deficiencies in humans.Update on clinical aspects and treatment of selected vitamin-responsive disorders II (riboflavin and CoQ 10).Drugs and mitochondrial diseases: 40 queries and answers.Genetic bases and clinical manifestations of coenzyme Q10 (CoQ 10) deficiency.Invertebrate models for coenzyme q10 deficiency.Border between natural product and drug: comparison of the related benzoquinones idebenone and coenzyme Q10 Fatal neonatal encephalopathy and lactic acidosis caused by a homozygous loss-of-function variant in COQ9.Molecular diagnosis of coenzyme Q10 deficiency.Characterization of CoQ₁₀ biosynthesis in fibroblasts of patients with primary and secondary CoQ₁₀ deficiency.Biochemical Assessment of Coenzyme Q10 Deficiency.The Value of Coenzyme Q10 Determination in Mitochondrial Patients.Impact of Chemical Analogs of 4-Hydroxybenzoic Acid on Coenzyme Q Biosynthesis: From Inhibition to Bypass of Coenzyme Q Deficiency.Rescue of primary ubiquinone deficiency due to a novel COQ7 defect using 2,4-dihydroxybensoic acid.The Role of Sulfide Oxidation Impairment in the Pathogenesis of Primary CoQ Deficiency.Mitochondrial respiration without ubiquinone biosynthesis.

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Treatment of CoQ(10) deficient fibroblasts with ubiquinone, CoQ analogs, and vitamin C: time- and compound-dependent effects

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2010 nî lūn-bûn

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2010 թուականի Յուլիսին հրատարակուած գիտական յօդուած

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2010年の論文

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Treatment of CoQ(10) deficient ...... and compound-dependent effects

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Treatment of CoQ(10) deficient ...... and compound-dependent effects

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Catarina M Quinzii

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P2860

A mutation in para-hydroxybenzoate-polyprenyl transferase (COQ2) causes primary coenzyme Q10 deficiency

A nonsense mutation in COQ9 causes autosomal-recessive neonatal-onset primary coenzyme Q10 deficiency: a potentially treatable form of mitochondrial disease

CABC1 gene mutations cause ubiquinone deficiency with cerebellar ataxia and seizures

ADCK3, an ancestral kinase, is mutated in a form of recessive ataxia associated with coenzyme Q10 deficiency

Prenyldiphosphate synthase, subunit 1 (PDSS1) and OH-benzoate polyprenyltransferase (COQ2) mutations in ubiquinone deficiency and oxidative phosphorylation disorders

A defect in coenzyme Q biosynthesis is responsible for the respiratory deficiency in Saccharomyces cerevisiae abc1 mutants.

Missense mutation of the COQ2 gene causes defects of bioenergetics and de novo pyrimidine synthesis

Interactions of mitochondria-targeted and untargeted ubiquinones with the mitochondrial respiratory chain and reactive oxygen species. Implications for the use of exogenous ubiquinones as therapies and experimental tools

Muscle coenzyme Q deficiency in familial mitochondrial encephalomyopathy

Phase II trial of CoQ10 for ALS finds insufficient evidence to justify phase III.

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