Redox signalling and mitochondrial stress responses; lessons from inborn errors of metabolism

Diet and exercise signals regulate SIRT3 and activate AMPK and PGC-1α in skeletal muscle

(2016) Orsolya M. Palacios et al.   Aging-US

Mitochondrial Biogenesis: Regulation By Endogenous Gases During Inflammation and Organ Stress

(2014) Hagir Suliman et al.   CURRENT PHARMACEUTICAL DESIGN

Mitochondrial Biogenesis in Health and Disease. Molecular and Therapeutic Approaches

(2014) Fabian Sanchis-Gomar et al.   CURRENT PHARMACEUTICAL DESIGN

Mitohormesis: Promoting Health and Lifespan by Increased Levels of Reactive Oxygen Species (ROS)

(2014) Michael Ristow et al.   Dose-Response

Redox regulation of antioxidants, autophagy, and the response to stress: Implications for electrophile therapeutics

(2014) Anna-Liisa Levonen et al.   FREE RADICAL BIOLOGY AND MEDICINE

AMPK: A cellular metabolic and redox sensor. A minireview

(2014) Najeeb Shirwany   Frontiers in Bioscience-Landmark

Cellular consequences of oxidative stress in riboflavin responsive multiple acyl-CoA dehydrogenation deficiency patient fibroblasts

(2014) N. Cornelius et al.   HUMAN MOLECULAR GENETICS

Frataxin deficiency increases cyclooxygenase 2 and prostaglandins in cell and animal models of Friedreich's ataxia

(2014) Genki Hayashi et al.   HUMAN MOLECULAR GENETICS

Sites of Superoxide and Hydrogen Peroxide Production by Muscle Mitochondria Assessedex Vivounder Conditions Mimicking Rest and Exercise

(2014) Renata L. S. Goncalves et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

No effect of bezafibrate in patients with CPTII and VLCAD deficiencies

(2014) Mette Cathrine Ørngreen et al.   JOURNAL OF INHERITED METABOLIC DISEASE

Mitochondrial allostatic load puts the 'gluc' back in glucocorticoids

(2014) Martin Picard et al.   Nature Reviews Endocrinology

Bezafibrate in skeletal muscle fatty acid oxidation disorders: A randomized clinical trial

(2014) M. C. Orngreen et al.   NEUROLOGY

Stilbenes and resveratrol metabolites improve mitochondrial fatty acid oxidation defects in human fibroblasts

(2014) Virginie Aires et al.   Orphanet Journal of Rare Diseases

Clinical and genetical heterogeneity of late-onset multiple acyl-coenzyme A dehydrogenase deficiency

(2014) Sarah C Grünert   Orphanet Journal of Rare Diseases

PGC-1α buffers ROS-mediated removal of mitochondria during myogenesis

(2014) S Baldelli et al.   Cell Death & Disease

HIF-1-Mediated Suppression of Acyl-CoA Dehydrogenases and Fatty Acid Oxidation Is Critical for Cancer Progression

(2014) De Huang et al.   Cell Reports

Hydrogen peroxide sensing, signaling and regulation of transcription factors

(2014) H. Susana Marinho et al.   Redox Biology

Lipid Replacement Therapy: A natural medicine approach to replacing damaged lipids in cellular membranes and organelles and restoring function

(2013) Garth L. Nicolson et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOMEMBRANES

Punctum on two different transcription factors regulated by PGC-1α: Nuclear factor erythroid-derived 2-like 2 and nuclear respiratory factor 2

(2013) Sara Baldelli et al.   BIOCHIMICA ET BIOPHYSICA ACTA-GENERAL SUBJECTS

Role of AMPK-mediated adaptive responses in human cells with mitochondrial dysfunction to oxidative stress

(2013) Shi-Bei Wu et al.   BIOCHIMICA ET BIOPHYSICA ACTA-GENERAL SUBJECTS

Mitochondrial quality control and communications with the nucleus are important in maintaining mitochondrial function and cell health

(2013) Vassilios N. Kotiadis et al.   BIOCHIMICA ET BIOPHYSICA ACTA-GENERAL SUBJECTS

Regulation of mitochondrial fatty acid β-oxidation in human: What can we learn from inborn fatty acid β-oxidation deficiencies?

(2013) Jean Bastin   BIOCHIMIE

Quality control of mitochondria during aging: Is there a good and a bad side of mitochondrial dynamics?

(2013) Marc Thilo Figge et al.   BIOESSAYS

Turn up the power - pharmacological activation of mitochondrial biogenesis in mouse models

(2013) J C Komen et al.   BRITISH JOURNAL OF PHARMACOLOGY

Antagonistic crosstalk between NF-κB and SIRT1 in the regulation of inflammation and metabolic disorders

(2013) Anu Kauppinen et al.   CELLULAR SIGNALLING

Divergent Mitochondrial Biogenesis Responses in Human Cardiomyopathy

(2013) Preeti Ahuja et al.   CIRCULATION

Genomic and non-genomic regulation of PGC1 isoforms by estrogen to increase cerebral vascular mitochondrial biogenesis and reactive oxygen species protection

(2013) Martin F. Kemper et al.   EUROPEAN JOURNAL OF PHARMACOLOGY

Secondary coenzyme Q10 deficiency and oxidative stress in cultured fibroblasts from patients with riboflavin responsive multiple Acyl-CoA dehydrogenation deficiency

(2013) N. Cornelius et al.   HUMAN MOLECULAR GENETICS

Beneficial effects of resveratrol on respiratory chain defects in patients' fibroblasts involve estrogen receptor and estrogen-related receptor alpha signaling

(2013) A. Lopes Costa et al.   HUMAN MOLECULAR GENETICS

Mitochondria and Reactive Oxygen Species: Physiology and Pathophysiology

(2013) Subhashini Bolisetty et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

Resveratrol Induces a Mitochondrial Complex I-dependent Increase in NADH Oxidation Responsible for Sirtuin Activation in Liver Cells

(2013) Valérie Desquiret-Dumas et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Current concepts in organic acidurias: understanding intra- and extracerebral disease manifestation

(2013) Stefan Kölker et al.   JOURNAL OF INHERITED METABOLIC DISEASE

Mitochondrial respiratory chain Complexes I and IV are impaired by β-amyloid via direct interaction and through Complex I-dependent ROS production, respectively

(2013) A. Bobba et al.   MITOCHONDRION

Dual control of mitochondrial biogenesis by sirtuin 1 and sirtuin 3

(2013) Julia Brenmoehl et al.   MITOCHONDRION

Mitochondrial retrograde signaling at the crossroads of tumor bioenergetics, genetics and epigenetics

(2013) Manti Guha et al.   MITOCHONDRION

Genetic and cellular modifiers of oxidative stress: What can we learn from fatty acid oxidation defects?

(2013) Rikke Katrine Jentoft Olsen et al.   MOLECULAR GENETICS AND METABOLISM

The nexus of chromatin regulation and intermediary metabolism

(2013) Philipp Gut et al.   NATURE

Riociguat for Pulmonary Hypertension — A Glass Half Full

(2013) Stephen L. Archer   NEW ENGLAND JOURNAL OF MEDICINE

Effect of Resveratrol on Cultured Skin Fibroblasts from Patients with Oxidative Phosphorylation Defects

(2013) Boel De Paepe et al.   PHYTOTHERAPY RESEARCH

Primary Respiratory Chain Disease Causes Tissue-Specific Dysregulation of the Global Transcriptome and Nutrient-Sensing Signaling Network

(2013) Zhe Zhang et al.   PLoS One

Anti-Inflammatory Therapy in Chronic Disease: Challenges and Opportunities

(2013) I. Tabas et al.   SCIENCE

Mitochondria and FOXO3: breath or die

(2013) Judith Hagenbuchner et al.   Frontiers in Physiology

Interaction of aldehydes derived from lipid peroxidation and membrane proteins

(2013) Stefania Pizzimenti et al.   Frontiers in Physiology

Glucose availability is a decisive factor for Nrf2-mediated gene expression

(2013) Elke H. Heiss et al.   Redox Biology

Upregulation of the mitochondrial Lon Protease allows adaptation to acute oxidative stress but dysregulation is associated with chronic stress, disease, and aging

(2013) Jenny K. Ngo et al.   Redox Biology

The biphasic redox sensing of SENP3 accounts for the HIF-1 transcriptional activity shift by oxidative stress

(2012) Ying Wang et al.   ACTA PHARMACOLOGICA SINICA

p53 Orchestrates the PGC-1α-Mediated Antioxidant Response Upon Mild Redox and Metabolic Imbalance

(2012) Katia Aquilano et al.   ANTIOXIDANTS & REDOX SIGNALING

Cell signalling by reactive lipid species: new concepts and molecular mechanisms

(2012) Ashlee Higdon et al.   BIOCHEMICAL JOURNAL

Mitochondria: In Sickness and in Health

(2012) Jodi Nunnari et al.   CELL

mTOR Signaling in Growth Control and Disease

(2012) Mathieu Laplante et al.   CELL

Coenzyme Q10 increases the fatty acid oxidation through AMPK-mediated PPARα induction in 3T3-L1 preadipocytes

(2012) Soo Kyung Lee et al.   CELLULAR SIGNALLING

OXPHOS mutations and neurodegeneration

(2012) Werner J H Koopman et al.   EMBO JOURNAL

Cardiac involvement in mitochondrial DNA disease: clinical spectrum, diagnosis, and management

(2012) M. G. D. Bates et al.   EUROPEAN HEART JOURNAL

Establishing cellular stress response profiles as biomarkers of homeodynamics, health and hormesis

(2012) Dino Demirovic et al.   EXPERIMENTAL GERONTOLOGY

Redox regulation of mitochondrial biogenesis

(2012) Claude A. Piantadosi et al.   FREE RADICAL BIOLOGY AND MEDICINE

Mechanism of superoxide and hydrogen peroxide generation by human electron-transfer flavoprotein and pathological variants

(2012) João V. Rodrigues et al.   FREE RADICAL BIOLOGY AND MEDICINE

Molecular mechanisms of riboflavin responsiveness in patients with ETF-QO variations and multiple acyl-CoA dehydrogenation deficiency

(2012) N. Cornelius et al.   HUMAN MOLECULAR GENETICS

Adaptive Response, Evidence of Cross-Resistance and Its Potential Clinical Use

(2012) Irina Milisav et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

Fueling the flame: bioenergy couples metabolism and inflammation

(2012) T. F. Liu et al.   JOURNAL OF LEUKOCYTE BIOLOGY

Curbing cancer's sweet tooth: Is there a role for MnSOD in regulation of the Warburg effect?

(2012) Aaron K. Holley et al.   MITOCHONDRION

Bezafibrate can be a new treatment option for mitochondrial fatty acid oxidation disorders: Evaluation by in vitro probe acylcarnitine assay

(2012) Seiji Yamaguchi et al.   MOLECULAR GENETICS AND METABOLISM

The many faces of insulin-like peptide signalling in the brain

(2012) Ana M. Fernandez et al.   NATURE REVIEWS NEUROSCIENCE

Effects of aerobic training on exercise-related oxidative stress in mitochondrial myopathies

(2012) Gabriele Siciliano et al.   NEUROMUSCULAR DISORDERS

Recovery of MERRF Fibroblasts and Cybrids Pathophysiology by Coenzyme Q10

(2012) Mario De la Mata et al.   Neurotherapeutics

Monogenic Mitochondrial Disorders

(2012) Werner J.H. Koopman et al.   NEW ENGLAND JOURNAL OF MEDICINE

Copper and bezafibrate cooperate to rescue cytochrome c oxidase deficiency in cells of patients with sco2 mutations

(2012) Alberto Casarin et al.   Orphanet Journal of Rare Diseases

Mitochondrial Fission, Fusion, and Stress

(2012) R. J. Youle et al.   SCIENCE

Mitochondrial quality control: an integrated network of pathways

(2012) Fabian Fischer et al.   TRENDS IN BIOCHEMICAL SCIENCES

Transcriptional integration of mitochondrial biogenesis

(2012) Richard C. Scarpulla et al.   TRENDS IN ENDOCRINOLOGY AND METABOLISM

PPAR and Oxidative Stress: Con() Catenating NRF2 and FOXO

(2012) Simone Polvani et al.   PPAR Research

AMPK-mediated increase of glycolysis as an adaptive response to oxidative stress in human cells: Implication of the cell survival in mitochondrial diseases

(2011) Shi-Bei Wu et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Cellular stress responses, hormetic phytochemicals and vitagenes in aging and longevity

(2011) Vittorio Calabrese et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Transcriptome analysis in mitochondrial disorders

(2011) Matthias Elstner et al.   BRAIN RESEARCH BULLETIN

SIRT3 Controls Cancer Metabolic Reprogramming by Regulating ROS and HIF

(2011) Paul T. Schumacker   CANCER CELL

SIRT3 Opposes Reprogramming of Cancer Cell Metabolism through HIF1α Destabilization

(2011) Lydia W.S. Finley et al.   CANCER CELL

Induction of the Mitochondrial NDUFA4L2 Protein by HIF-1α Decreases Oxygen Consumption by Inhibiting Complex I Activity

(2011) Daniel Tello et al.   Cell Metabolism

Activation of stress signaling pathways by electrophilic oxidized and nitrated lipids

(2011) Emilia Kansanen et al.   FREE RADICAL BIOLOGY AND MEDICINE

Mitochondrial respiratory complex I dysfunction promotes tumorigenesis through ROS alteration and AKT activation

(2011) Lokendra Kumar Sharma et al.   HUMAN MOLECULAR GENETICS

Disruption of mitochondrial homeostasis in organic acidurias: insights from human and animal studies

(2011) Moacir Wajner et al.   JOURNAL OF BIOENERGETICS AND BIOMEMBRANES

Exercise Increases Mitochondrial PGC-1α Content and Promotes Nuclear-Mitochondrial Cross-talk to Coordinate Mitochondrial Biogenesis

(2011) Adeel Safdar et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

AMP-activated protein kinase inhibits NF-κB signaling and inflammation: impact on healthspan and lifespan

(2011) Antero Salminen et al.   JOURNAL OF MOLECULAR MEDICINE-JMM

Characterization of mitochondrial proteome in a severe case of ETF-QO deficiency

(2011) H. Rocha et al.   Journal of Proteomics

SirT3 suppresses hypoxia inducible factor 1α and tumor growth by inhibiting mitochondrial ROS production

(2011) E L Bell et al.   ONCOGENE

Vulnerability to Oxidative Stress In Vitro in Pathophysiology of Mitochondrial Short-Chain Acyl-CoA Dehydrogenase Deficiency: Response to Antioxidants

(2011) Zarazuela Zolkipli et al.   PLoS One

Screening for Active Small Molecules in Mitochondrial Complex I Deficient Patient's Fibroblasts, Reveals AICAR as the Most Beneficial Compound

(2011) Anna Golubitzky et al.   PLoS One

Age-dependent cardiomyopathy in mitochondrial mutator mice is attenuated by overexpression of catalase targeted to mitochondria

(2010) Dao-Fu Dai et al.   AGING CELL

Superoxide dismutase downregulation in osteoarthritis progression and end-stage disease

(2010) J. L. Scott et al.   ANNALS OF THE RHEUMATIC DISEASES

The electron transfer flavoprotein: Ubiquinone oxidoreductases

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

Long-Term Follow-Up of Bezafibrate Treatment in Patients With the Myopathic Form of Carnitine Palmitoyltransferase 2 Deficiency

(2010) J P Bonnefont et al.   CLINICAL PHARMACOLOGY & THERAPEUTICS

Elevated free fatty acids and impaired adiponectin bioactivity contribute to reduced SOD2 protein in monocytes of type 2 diabetes patients

(2010) Sabrina Bauer et al.   EXPERIMENTAL AND MOLECULAR PATHOLOGY

Manganese superoxide dismutase dimorphism relationship with severity and prognosis in cardiogenic shock due to dilated cardiomyopathy

(2010) J. C. Charniot et al.   FREE RADICAL RESEARCH

p62/SQSTM1Is a Target Gene for Transcription Factor NRF2 and Creates a Positive Feedback Loop by Inducing Antioxidant Response Element-driven Gene Transcription

(2010) Ashish Jain et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Functional characterization of the promoter of the human Lon protease gene

(2010) Marcello Pinti et al.   MITOCHONDRION

Sirtuin 1 Modulates Cellular Responses to Hypoxia by Deacetylating Hypoxia-Inducible Factor 1α

(2010) Ji-Hong Lim et al.   MOLECULAR CELL

Mitochondrial DNA Mutation-Elicited Oxidative Stress, Oxidative Damage, and Altered Gene Expression in Cultured Cells of Patients with MERRF Syndrome

(2010) Shi-Bei Wu et al.   MOLECULAR NEUROBIOLOGY

A role for mitochondria in NLRP3 inflammasome activation

(2010) Rongbin Zhou et al.   NATURE

Circulating mitochondrial DAMPs cause inflammatory responses to injury

(2010) Qin Zhang et al.   NATURE

Expression of neutrophil SOD2 is reduced after lipopolysaccharide stimulation: a potential cause of neutrophil dysfunction in chronic kidney disease

(2010) J. Olsson et al.   NEPHROLOGY DIALYSIS TRANSPLANTATION

Superoxide Dismutase and Nicotinamide Adenine Dinucleotide Phosphate

(2010) Beatrice Mohelnikova-Duchonova et al.   PANCREAS

PGC-1alpha Down-Regulation Affects the Antioxidant Response in Friedreich's Ataxia

(2010) Daniele Marmolino et al.   PLoS One

Metabolism Is Not Boring

(2010) L. B. Ray   SCIENCE

Effect of heat stress and bezafibrate on mitochondrial β-oxidation: Comparison between cultured cells from normal and mitochondrial fatty acid oxidation disorder children using in vitro probe acylcarnitine profiling assay

(2009) Hong Li et al.   BRAIN & DEVELOPMENT

Activation of the AMPK-FOXO3 Pathway Reduces Fatty Acid-Induced Increase in Intracellular Reactive Oxygen Species by Upregulating Thioredoxin

(2009) X.-N. Li et al.   DIABETES

Mutations in the C. elegans Succinate Dehydrogenase Iron–Sulfur Subunit Promote Superoxide Generation and Premature Aging

(2009) Jingzhou Huang et al.   JOURNAL OF MOLECULAR BIOLOGY

Riboflavin-responsive lipid-storage myopathy caused by ETFDH gene mutations

(2009) B. Wen et al.   JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY

Energetics, epigenetics, mitochondrial genetics

(2009) Douglas C. Wallace et al.   MITOCHONDRION

Bezafibrate for an Inborn Mitochondrial Beta-Oxidation Defect

(2009) Jean-Paul Bonnefont et al.   NEW ENGLAND JOURNAL OF MEDICINE

Mechanisms Underlying Metabolic and Neural Defects in Zebrafish and Human Multiple Acyl-CoA Dehydrogenase Deficiency (MADD)

(2009) Yuanquan Song et al.   PLoS One

PINK1-dependent recruitment of Parkin to mitochondria in mitophagy

(2009) C. Vives-Bauza et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Understanding the Warburg Effect: The Metabolic Requirements of Cell Proliferation

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

Regulation of the aging process by autophagy

(2009) Antero Salminen et al.   TRENDS IN MOLECULAR MEDICINE

FOXO3A Regulates Peroxiredoxin III Expression in Human Cardiac Fibroblasts

(2008) Calin B. Chiribau et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Mitochondrial fatty acid oxidation defects—remaining challenges

(2008) Niels Gregersen et al.   JOURNAL OF INHERITED METABOLIC DISEASE

Inactivation of brain mitochondrial Lon protease by peroxynitrite precedes electron transport chain dysfunction

(2008) Lee Stanyer et al.   NEUROCHEMISTRY INTERNATIONAL

Leber hereditary optic neuropathy mutations in the ND6 subunit of mitochondrial complex I affect ubiquinone reduction kinetics in a bacterial model of the enzyme

(2007) Jukka Pätsi et al.   BIOCHEMICAL JOURNAL

Hypoxia-inducible Factor-1α Stabilization in Nonhypoxic Conditions: Role of Oxidation and Intracellular Ascorbate Depletion

(2007) Elisabeth L. Pagé et al.   MOLECULAR BIOLOGY OF THE CELL