Metabolic Targets of Coenzyme Q10 in Mitochondria

Pathogenic variants in SQOR encoding sulfide:quinone oxidoreductase are a potentially treatable cause of Leigh disease

(2020) Marisa W. Friederich et al.   JOURNAL OF INHERITED METABOLIC DISEASE

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

(2020) Pilar González-García et al.   HUMAN MOLECULAR GENETICS

Acid enhancement of ROS generation by complex-I reverse electron transport is balanced by acid inhibition of complex-II: Relevance for tissue reperfusion injury

(2020) Alexander S. Milliken et al.   Redox Biology

Fibroblast Growth Factor 21 Drives Dynamics of Local and Systemic Stress Responses in Mitochondrial Myopathy with mtDNA Deletions

(2019) Saara Forsström et al.   Cell Metabolism

FSP1 is a glutathione-independent ferroptosis suppressor

(2019) Sebastian Doll et al.   NATURE

The CoQ oxidoreductase FSP1 acts parallel to GPX4 to inhibit ferroptosis

(2019) Kirill Bersuker et al.   NATURE

The Paradox of Coenzyme Q10 in Aging

(2019) Díaz-Casado et al.   Nutrients

Bioenergetic consequences from xenotopic expression of a tunicate AOX in mouse mitochondria: Switch from RET and ROS to FET

(2019) Marten Szibor et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

The Complexity of Making Ubiquinone

(2019) Ying Wang et al.   TRENDS IN ENDOCRINOLOGY AND METABOLISM

Clinical syndromes associated with Coenzyme Q 10 deficiency

(2018) María Alcázar-Fabra et al.   Essays in Biochemistry

Coenzyme Q 10 deficiencies: pathways in yeast and humans

(2018) Agape M. Awad et al.   Essays in Biochemistry

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

(2018) Daniel J Fazakerley et al.   eLife

CoQ10 supplementation rescues nephrotic syndrome through normalization of H2S oxidation pathway

(2018) Giulio Kleiner et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Reactivation of Dihydroorotate Dehydrogenase-Driven Pyrimidine Biosynthesis Restores Tumor Growth of Respiration-Deficient Cancer Cells

(2018) Martina Bajzikova et al.   Cell Metabolism

β‐RA reduces DMQ/CoQ ratio and rescues the encephalopathic phenotype in Coq9R239X mice

(2018) Agustín Hidalgo‐Gutiérrez et al.   EMBO Molecular Medicine

Pathogenicity of two COQ7 mutations and responses to 2,4-dihydroxybenzoate bypass treatment

(2017) Ying Wang et al.   JOURNAL OF CELLULAR AND MOLECULAR MEDICINE

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

(2017) Inge Kühl et al.   eLife

Role of Mitochondrial Reverse Electron Transport in ROS Signaling: Potential Roles in Health and Disease

(2017) Filippo Scialò et al.   Frontiers in Physiology

Coenzyme Q biosynthesis and its role in the respiratory chain structure

(2016) María Alcázar-Fabra et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

Succinate Dehydrogenase Supports Metabolic Repurposing of Mitochondria to Drive Inflammatory Macrophages

(2016) Evanna L. Mills et al.   CELL

Mitochondrial DNA Replication Defects Disturb Cellular dNTP Pools and Remodel One-Carbon Metabolism

(2016) Joni Nikkanen et al.   Cell Metabolism

Mitochondrial ROS Produced via Reverse Electron Transport Extend Animal Lifespan

(2016) Filippo Scialò et al.   Cell Metabolism

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

(2016) Marta Luna‐Sánchez et al.   EMBO Molecular Medicine

Coenzyme Q deficiency causes impairment of the sulfide oxidation pathway

(2016) Marcello Ziosi et al.   EMBO Molecular Medicine

Gene Therapy Corrects Mitochondrial Dysfunction in Hematopoietic Progenitor Cells and Fibroblasts from Coq9R239X Mice

(2016) Eliana Barriocanal-Casado et al.   PLoS One

Understanding Ubiquinone

(2016) Ying Wang et al.   TRENDS IN CELL BIOLOGY

Altered intestinal microbiota–host mitochondria crosstalk in new onset Crohn’s disease

(2016) Walid Mottawea et al.   Nature Communications

The CoQH2/CoQ Ratio Serves as a Sensor of Respiratory Chain Efficiency

(2016) Adela Guarás et al.   Cell Reports

Mitochondrial dysfunction remodels one-carbon metabolism in human cells

(2016) Xiaoyan Robert Bao et al.   eLife

Proline dehydrogenase 2 (PRODH2) is a hydroxyproline dehydrogenase (HYPDH) and molecular target for treating primary hyperoxaluria

(2015) Candice B. Summitt et al.   BIOCHEMICAL JOURNAL

Biosynthesis of coenzyme Q in eukaryotes

(2015) Makoto Kawamukai   BIOSCIENCE BIOTECHNOLOGY AND BIOCHEMISTRY

Endogenous Hydrogen Sulfide Production Is Essential for Dietary Restriction Benefits

(2015) Christopher Hine et al.   CELL

Oxygen Sensing by Arterial Chemoreceptors Depends on Mitochondrial Complex I Signaling

(2015) M. Carmen Fernández-Agüera et al.   Cell Metabolism

ROS-dependent signal transduction

(2015) Colleen R Reczek et al.   CURRENT OPINION IN CELL BIOLOGY

Mitochondrial diseases caused by toxic compound accumulation: from etiopathology to therapeutic approaches

(2015) I. Di Meo et al.   EMBO Molecular Medicine

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

(2015) M. Luna-Sanchez et al.   EMBO Molecular Medicine

Calorie restriction and methionine restriction in control of endogenous hydrogen sulfide production by the transsulfuration pathway

(2015) Christopher Hine et al.   EXPERIMENTAL GERONTOLOGY

H 2 S: A Novel Gasotransmitter that Signals by Sulfhydration

(2015) Bindu D. Paul et al.   TRENDS IN BIOCHEMICAL SCIENCES

Mitochondrial function and lifespan of mice with controlled ubiquinone biosynthesis

(2015) Ying Wang et al.   Nature Communications

Sulfur as a Signaling Nutrient Through Hydrogen Sulfide

(2014) Omer Kabil et al.   Annual Review of Nutrition

Ubiquinol-10 ameliorates mitochondrial encephalopathy associated with CoQ deficiency

(2014) Laura García-Corzo et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Organization of the Human Mitochondrial Hydrogen Sulfide Oxidation Pathway

(2014) Marouane Libiad et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Genetic bases and clinical manifestations of coenzyme Q10 (CoQ10) deficiency

(2014) Maria Andrea Desbats et al.   JOURNAL OF INHERITED METABOLIC DISEASE

Ischaemic accumulation of succinate controls reperfusion injury through mitochondrial ROS

(2014) Edward T. Chouchani et al.   NATURE

Mitochondrial COQ9 is a lipid-binding protein that associates with COQ7 to enable coenzyme Q biosynthesis

(2014) D. C. Lohman et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Transcriptional and metabolic adaptation of human neurons to the mitochondrial toxicant MPP+

(2014) A K Krug et al.   Cell Death & Disease

Hypoxia-Inducible Factor 3 Is an Oxygen-Dependent Transcription Activator and Regulates a Distinct Transcriptional Response to Hypoxia

(2014) Peng Zhang et al.   Cell Reports

Ubiad1 Is an Antioxidant Enzyme that Regulates eNOS Activity by CoQ10 Synthesis

(2013) Vera Mugoni et al.   CELL

Coenzyme Q10 deficiency in mitochondrial DNA depletion syndromes

(2013) Raquel Montero et al.   MITOCHONDRION

Supercomplex Assembly Determines Electron Flux in the Mitochondrial Electron Transport Chain

(2013) E. Lapuente-Brun et al.   SCIENCE

Tissue-specific oxidative stress and loss of mitochondria in CoQ-deficient Pdss2 mutant mice

(2012) Catarina M. Quinzii et al.   FASEB JOURNAL

Intramitochondrial hydrogen sulfide production by 3-mercaptopyruvate sulfurtransferase maintains mitochondrial electron flow and supports cellular bioenergetics

(2012) Katalin Módis et al.   FASEB JOURNAL

Dysfunctional Coq9 protein causes predominant encephalomyopathy associated with CoQ deficiency

(2012) L. Garcia-Corzo et al.   HUMAN MOLECULAR GENETICS

Prerequisites for ubiquinone analogs to prevent mitochondrial permeability transition-induced cell death

(2012) Julie Belliere et al.   JOURNAL OF BIOENERGETICS AND BIOMEMBRANES

Human neuronal coenzyme Q10 deficiency results in global loss of mitochondrial respiratory chain activity, increased mitochondrial oxidative stress and reversal of ATP synthase activity: implications for pathogenesis and treatment

(2012) Kate E. C. Duberley et al.   JOURNAL OF INHERITED METABOLIC DISEASE

Steady-state kinetic mechanism of the proline:ubiquinone oxidoreductase activity of proline utilization A (PutA) from Escherichia coli

(2011) Michael A. Moxley et al.   ARCHIVES OF BIOCHEMISTRY AND BIOPHYSICS

Mitochondrial H2O2 generated from electron transport chain complex I stimulates muscle differentiation

(2011) Seonmin Lee et al.   CELL RESEARCH

The role of DMQ9 in the long-lived mutant clk-1

(2011) Yu-Ying Yang et al.   MECHANISMS OF AGEING AND DEVELOPMENT

Increased transsulfuration mediates longevity and dietary restriction in Drosophila

(2011) H. Kabil et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

The electron transfer flavoprotein: Ubiquinone oxidoreductases

(2010) Nicholas J. Watmough et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

Supramolecular organizations in the aerobic respiratory chain of Escherichia coli

(2010) Pedro M.F. Sousa et al.   BIOCHIMIE

Reactive oxygen species, oxidative stress, and cell death correlate with level of CoQ10 deficiency

(2010) Catarina M. Quinzii et al.   FASEB JOURNAL

Mitochondrial myopathy induces a starvation-like response

(2010) Henna Tyynismaa et al.   HUMAN MOLECULAR GENETICS

Treatment of CoQ10 Deficient Fibroblasts with Ubiquinone, CoQ Analogs, and Vitamin C: Time- and Compound-Dependent Effects

(2010) Luis C. López et al.   PLoS One

Ubiquinone Analogs: A Mitochondrial Permeability Transition Pore-Dependent Pathway to Selective Cell Death

(2010) Flavien Devun et al.   PLoS One

A Nonsense Mutation in COQ9 Causes Autosomal-Recessive Neonatal-Onset Primary Coenzyme Q10 Deficiency: A Potentially Treatable Form of Mitochondrial Disease

(2009) Andrew J. Duncan et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Loss of ETHE1, a mitochondrial dioxygenase, causes fatal sulfide toxicity in ethylmalonic encephalopathy

(2009) Valeria Tiranti et al.   NATURE MEDICINE

ETFDH mutations, CoQ10 levels, and respiratory chain activities in patients with riboflavin-responsive multiple acyl-CoA dehydrogenase deficiency

(2009) Wen-Chen Liang et al.   NEUROMUSCULAR DISORDERS

Coenzyme Q10 deficiency associated with a mitochondrial DNA depletion syndrome: A case report

(2008) Raquel Montero et al.   CLINICAL BIOCHEMISTRY

Respiratory chain dysfunction and oxidative stress correlate with severity of primary CoQ10 deficiency

(2008) Catarina M. Quinzii et al.   FASEB JOURNAL

Three enzymatic activities catalyze the oxidation of sulfide to thiosulfate in mammalian and invertebrate mitochondria

(2008) Tatjana M. Hildebrandt et al.   FEBS Journal

Respiratory Active Mitochondrial Supercomplexes

(2008) Rebeca Acín-Pérez et al.   MOLECULAR CELL