Minimal mitochondrial respiration is required to prevent cell death by inhibition of mTOR signaling in CoQ-deficient cells

mTOR at the nexus of nutrition, growth, ageing and disease

(2020) Grace Y. Liu et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

Micellization of coenzyme Q by the fungicide caspofungin allows for safe intravenous administration to reach extreme supraphysiological concentrations

(2020) Ying Wang et al.   Redox Biology

The good, the bad and the autophagosome: exploring unanswered questions of autophagy-dependent cell death

(2019) Jurgen Kriel et al.   CELL DEATH AND DIFFERENTIATION

Rapamycin administration is not a valid therapeutic strategy for every case of mitochondrial disease

(2019) Eliana Barriocanal-Casado et al.   EBioMedicine

Drug Development for the Therapy of Mitochondrial Diseases

(2019) Volkmar Weissig   TRENDS IN MOLECULAR MEDICINE

The Complexity of Making Ubiquinone

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

Emerging therapies for mitochondrial diseases

(2018) Michio Hirano et al.   Essays in Biochemistry

Rapamycin rescues mitochondrial myopathy via coordinated activation of autophagy and lysosomal biogenesis

(2018) Gabriele Civiletto et al.   EMBO Molecular Medicine

Discovery and Characterization of AZD6738, a Potent Inhibitor of Ataxia Telangiectasia Mutated and Rad3 Related (ATR) Kinase with Application as an Anticancer Agent

(2018) Kevin M. Foote et al.   JOURNAL OF MEDICINAL CHEMISTRY

Low-dose rapamycin extends lifespan in a mouse model of mtDNA depletion syndrome

(2017) Stephanie E Siegmund et al.   HUMAN MOLECULAR GENETICS

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

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

An Anti-Cancer Drug Candidate OSI-027 and its Analog as Inhibitors of mTOR: Computational Insights Into the Inhibitory Mechanisms

(2017) Mohd Rehan   JOURNAL OF CELLULAR BIOCHEMISTRY

Glucose Deprivation Induces ATF4-Mediated Apoptosis through TRAIL Death Receptors

(2017) Raffaella Iurlaro et al.   MOLECULAR AND CELLULAR BIOLOGY

Biochemistry of Mitochondrial Coenzyme Q Biosynthesis

(2017) Jonathan A. Stefely et al.   TRENDS IN BIOCHEMICAL SCIENCES

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

(2017) Inge Kühl et al.   eLife

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

(2017) Bryan G. Hughes et al.   Scientific Reports

Mammalian Autophagy: How Does It Work?

(2016) Carla F. Bento et al.   Annual Review of Biochemistry

Modifying the Mitochondrial Genome

(2016) Alexander N. Patananan et al.   Cell Metabolism

Cerebellar ataxia and severe muscle CoQ10deficiency in a patient with a novel mutation inADCK3

(2016) E. Barca et al.   CLINICAL GENETICS

Secondary coenzyme Q 10 deficiencies in oxidative phosphorylation (OXPHOS) and non-OXPHOS disorders

(2016) Delia Yubero et al.   MITOCHONDRION

Alleviation of neuronal energy deficiency by mTOR inhibition as a treatment for mitochondria-related neurodegeneration

(2016) Xinde Zheng et al.   eLife

COQ4 Mutations Cause a Broad Spectrum of Mitochondrial Disorders Associated with CoQ10 Deficiency

(2015) Gloria Brea-Calvo et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Molecular Pathways: Targeting ATR in Cancer Therapy

(2015) L. M. Karnitz et al.   CLINICAL CANCER RESEARCH

Inhibiting cytosolic translation and autophagy improves health in mitochondrial disease

(2015) Min Peng et al.   HUMAN MOLECULAR GENETICS

Rescue of primary ubiquinone deficiency due to a novelCOQ7defect using 2,4–dihydroxybensoic acid

(2015) Christoph Freyer et al.   JOURNAL OF MEDICAL GENETICS

Mitochondrial function and lifespan of mice with controlled ubiquinone biosynthesis

(2015) Ying Wang et al.   Nature Communications

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

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

Mitochondrial respiration without ubiquinone biosynthesis

(2013) Ying Wang et al.   HUMAN MOLECULAR GENETICS

Glutamine and cancer: cell biology, physiology, and clinical opportunities

(2013) Christopher T. Hensley et al.   JOURNAL OF CLINICAL INVESTIGATION

ADCK4 mutations promote steroid-resistant nephrotic syndrome through CoQ10 biosynthesis disruption

(2013) Shazia Ashraf et al.   JOURNAL OF CLINICAL INVESTIGATION

A novel mutation in COQ2 leading to fatal infantile multisystem disease

(2013) Bernadette S. Jakobs et al.   JOURNAL OF THE NEUROLOGICAL SCIENCES

Coenzyme Q10 deficiency in mitochondrial DNA depletion syndromes

(2013) Raquel Montero et al.   MITOCHONDRION

mTOR Inhibition Alleviates Mitochondrial Disease in a Mouse Model of Leigh Syndrome

(2013) S. C. Johnson et al.   SCIENCE

Heterogeneity of Coenzyme Q10Deficiency

(2012) Valentina Emmanuele et al.   ARCHIVES OF NEUROLOGY

mTOR Signaling in Growth Control and Disease

(2012) Mathieu Laplante et al.   CELL

Glucose-Independent Glutamine Metabolism via TCA Cycling for Proliferation and Survival in B Cells

(2012) Anne Le et al.   Cell Metabolism

Molecular genetics of ubiquinone biosynthesis in animals

(2012) Ying Wang et al.   CRITICAL REVIEWS IN BIOCHEMISTRY AND MOLECULAR BIOLOGY

Dysfunctional Coq9 protein causes predominant encephalomyopathy associated with CoQ deficiency

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

Haploinsufficiency ofCOQ4causes coenzyme Q10deficiency

(2012) Leonardo Salviati et al.   JOURNAL OF MEDICAL GENETICS

Coenzyme Q10 deficiency in patients with Parkinson's disease

(2012) Laurie K. Mischley et al.   JOURNAL OF THE NEUROLOGICAL SCIENCES

Primary and secondary CoQ10 deficiencies in humans

(2011) Catarina M. Quinzii et al.   BIOFACTORS

COQ6 mutations in human patients produce nephrotic syndrome with sensorineural deafness

(2011) Saskia F. Heeringa et al.   JOURNAL OF CLINICAL INVESTIGATION

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

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

Preclinical Characterization of OSI-027, a Potent and Selective Inhibitor of mTORC1 and mTORC2: Distinct from Rapamycin

(2011) S. V. Bhagwat et al.   MOLECULAR CANCER THERAPEUTICS

Cerebellar defects in Pdss2 conditional knockout mice during embryonic development and in adulthood

(2011) Song Lu et al.   NEUROBIOLOGY OF DISEASE

Decreased Energy Metabolism Extends Life Span in Caenorhabditis elegans Without Reducing Oxidative Damage

(2010) J. M. Van Raamsdonk et al.   GENETICS

Glucose Addiction of TSC Null Cells Is Caused by Failed mTORC1-Dependent Balancing of Metabolic Demand with Supply

(2010) Andrew Y. Choo et al.   MOLECULAR CELL

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

Glioblastoma Cells Require Glutamate Dehydrogenase to Survive Impairments of Glucose Metabolism or Akt Signaling

(2009) C. Yang et al.   CANCER RESEARCH

An ATP-competitive Mammalian Target of Rapamycin Inhibitor Reveals Rapamycin-resistant Functions of mTORC1

(2009) Carson C. Thoreen et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Coenzyme Q10 is frequently reduced in muscle of patients with mitochondrial myopathy

(2009) Sabrina Sacconi et al.   NEUROMUSCULAR DISORDERS

Glucocorticoids increase NPY gene expression in the arcuate nucleus by inhibiting mTOR signaling in rat hypothalamic organotypic cultures

(2009) Hiroshi Shimizu et al.   PEPTIDES

Understanding the Warburg Effect: The Metabolic Requirements of Cell Proliferation

(2009) M. G. Vander Heiden et al.   SCIENCE

ADCK3, an Ancestral Kinase, Is Mutated in a Form of Recessive Ataxia Associated with Coenzyme Q10 Deficiency

(2008) Clotilde Lagier-Tourenne et al.   AMERICAN JOURNAL OF HUMAN GENETICS

CABC1 Gene Mutations Cause Ubiquinone Deficiency with Cerebellar Ataxia and Seizures

(2008) Julie Mollet et al.   AMERICAN JOURNAL OF HUMAN GENETICS

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

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