Pharmacological advances in mitochondrial therapy

Metabolic effects of bezafibrate in mitochondrial disease

(2020) Hannah Steele et al.   EMBO Molecular Medicine

Patient reported outcomes in Friedreich's Ataxia after withdrawal from Idebenone

(2019) Arron Cook et al.   ACTA NEUROLOGICA SCANDINAVICA

Mdivi-1, a mitochondrial fission inhibitor, modulates T helper cells and suppresses the development of experimental autoimmune encephalomyelitis

(2019) Yan-Hua Li et al.   Journal of Neuroinflammation

An old medicine as a new drug to prevent mitochondrial complex I from producing oxygen radicals

(2019) Dominique Detaille et al.   PLoS One

Mitochondrial Dysfunction Plays a Key Role in the Development of Neurodegenerative Diseases in Diabetes

(2019) Han Cheng et al.   AMERICAN JOURNAL OF PHYSIOLOGY-ENDOCRINOLOGY AND METABOLISM

P4607A specific complex I-induced ROS modulator, OP2113, is a new cardioproctective agent against acute myocardial infarction injuries during reperfusion

(2019) A Boucard et al.   EUROPEAN HEART JOURNAL

Insulin sensitizer MSDC-0602K in non-alcoholic steatohepatitis: A randomized, double-blind, placebo-controlled phase IIb study

(2019) Stephen A. Harrison et al.   JOURNAL OF HEPATOLOGY

Long-term data with idebenone on respiratory function outcomes in patients with Duchenne muscular dystrophy

(2019) Laurent Servais et al.   NEUROMUSCULAR DISORDERS

Mitochondrial dynamic modulation exerts cardiometabolic protection in obese insulin-resistant rats

(2019) Chayodom Maneechote et al.   CLINICAL SCIENCE

Response to Pioglitazone in Patients With Nonalcoholic Steatohepatitis With vs Without Type 2 Diabetes

(2018) Fernando Bril et al.   Clinical Gastroenterology and Hepatology

Imeglimin Protects INS-1 Cells and Human Islets against High Glucose– and High Fructose–Induced Cell Death by Inhibiting the Mitochondrial PTP Opening

(2018) SANDRINE LABLANCHE et al.   DIABETES

Inhibition of Drp1/Fis1 interaction slows progression of amyotrophic lateral sclerosis

(2018) Amit U Joshi et al.   EMBO Molecular Medicine

Role of mitochondrial ROS in the brain: from physiology to neurodegeneration

(2018) Plamena R. Angelova et al.   FEBS LETTERS

Targeting energy metabolism via the mitochondrial pyruvate carrier as a novel approach to attenuate neurodegeneration

(2018) Emmanuel Quansah et al.   Molecular Neurodegeneration

MFN2 agonists reverse mitochondrial defects in preclinical models of Charcot-Marie-Tooth disease type 2A

(2018) Agostinho G. Rocha et al.   SCIENCE

Oxidative stress, aging, and diseases

(2018) Ilaria Liguori et al.   Clinical Interventions in Aging

Idebenone: Novel Strategies to Improve Its Systemic and Local Efficacy

(2018) Lucia Montenegro et al.   Nanomaterials

The KHENERGY Study: Safety and Efficacy of KH176 in Mitochondrial m.3243A>G Spectrum Disorders

(2018) Mirian C.H. Janssen et al.   CLINICAL PHARMACOLOGY & THERAPEUTICS

Differential temporal inhibition of mitochondrial fission by Mdivi-1 exerts effective cardioprotection in cardiac ischemia/reperfusion injury

(2018) Chayodom Maneechote et al.   CLINICAL SCIENCE

Mitochondria as oncotarget: a comparison between the tetracycline analogs doxycycline and COL-3

(2018) Margherita Protasoni et al.   Oncotarget

KH176 Safeguards Mitochondrial Diseased Cells from Redox Stress-Induced Cell Death by Interacting with the Thioredoxin System/Peroxiredoxin Enzyme Machinery

(2018) Julien Beyrath et al.   Scientific Reports

Antidiabetic potential of saffron and its active constituents

(2018) Habib Yaribeygi et al.   JOURNAL OF CELLULAR PHYSIOLOGY

Inhibition of mitochondrial translation as a therapeutic strategy for human ovarian cancer to overcome chemoresistance

(2018) Bo Hu et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

Targeting the mitochondrial pyruvate carrier attenuates fibrosis in a mouse model of nonalcoholic steatohepatitis

(2017) Kyle S. McCommis et al.   HEPATOLOGY

Resveratrol regulates neuro-inflammation and induces adaptive immunity in Alzheimer’s disease

(2017) Charbel Moussa et al.   Journal of Neuroinflammation

Inhibition of Drp1 Ameliorates Synaptic Depression, Aβ Deposition, and Cognitive Impairment in an Alzheimer's Disease Model

(2017) Seung Hyun Baek et al.   JOURNAL OF NEUROSCIENCE

Therapies for mitochondrial diseases and current clinical trials

(2017) Ayman W. El-Hattab et al.   MOLECULAR GENETICS AND METABOLISM

Glitazone use associated with reduced risk of Parkinson's disease

(2017) Brage Brakedal et al.   MOVEMENT DISORDERS

AMPK: guardian of metabolism and mitochondrial homeostasis

(2017) Sébastien Herzig et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

Targeting mitochondrial translation by inhibiting DDX3: a novel radiosensitization strategy for cancer treatment

(2017) M R Heerma van Voss et al.   ONCOGENE

KH176 under development for rare mitochondrial disease: a first in man randomized controlled clinical trial in healthy male volunteers

(2017) Saskia Koene et al.   Orphanet Journal of Rare Diseases

Drp1/Fis1 interaction mediates mitochondrial dysfunction, bioenergetic failure and cognitive decline in Alzheimer's disease

(2017) Amit U. Joshi et al.   Oncotarget

Long-Term Pioglitazone Treatment for Patients With Nonalcoholic Steatohepatitis and Prediabetes or Type 2 Diabetes Mellitus

(2016) Kenneth Cusi et al.   ANNALS OF INTERNAL MEDICINE

Mito-Morphosis: Mitochondrial Fusion, Fission, and Cristae Remodeling as Key Mediators of Cellular Function

(2016) Lena Pernas et al.   Annual Review of Physiology

The mitochondrial pyruvate carrier in health and disease: To carry or not to carry?

(2016) Tom Bender et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

The process of mammalian mitochondrial protein synthesis

(2016) Nicole Mai et al.   CELL AND TISSUE RESEARCH

Differential contribution of the mitochondrial translation pathway to the survival of diffuse large B-cell lymphoma subsets

(2016) Erik Norberg et al.   CELL DEATH AND DIFFERENTIATION

Mitochondrial biogenesis and clearance: a balancing act

(2016) Christina Ploumi et al.   FEBS Journal

Tigecycline targets nonsmall cell lung cancer through inhibition of mitochondrial function

(2016) Xuefeng Jia et al.   FUNDAMENTAL & CLINICAL PHARMACOLOGY

Sirt1 and the Mitochondria

(2016)   MOLECULES AND CELLS

Mitochondrial pyruvate carrier regulates autophagy, inflammation, and neurodegeneration in experimental models of Parkinsons disease

(2016) A. Ghosh et al.   Science Translational Medicine

Inhibition of Drp1 mitochondrial translocation provides neural protection in dopaminergic system in a Parkinson’s disease model induced by MPTP

(2016) Emily Filichia et al.   Scientific Reports

Activation of sirtuin 1 as therapy for the peroxisomal disease adrenoleukodystrophy

(2015) L Morató et al.   CELL DEATH AND DIFFERENTIATION

Mitochondrial pyruvate import and its effects on homeostasis

(2015) Benoît Vanderperre et al.   CURRENT OPINION IN CELL BIOLOGY

An open-label trial in Friedreich ataxia suggests clinical benefit with high-dose resveratrol, without effect on frataxin levels

(2015) Eppie M. Yiu et al.   JOURNAL OF NEUROLOGY

Efficacy of idebenone on respiratory function in patients with Duchenne muscular dystrophy not using glucocorticoids (DELOS): a double-blind randomised placebo-controlled phase 3 trial

(2015) Gunnar M Buyse et al.   LANCET

Pioglitazone in early Parkinson's disease: a phase 2, multicentre, double-blind, randomised trial

(2015)   LANCET NEUROLOGY

RNA helicase DDX3: a novel therapeutic target in Ewing sarcoma

(2015) B A Wilky et al.   ONCOGENE

NAD+-Dependent Activation of Sirt1 Corrects the Phenotype in a Mouse Model of Mitochondrial Disease

(2014) Raffaele Cerutti et al.   Cell Metabolism

An Evaluation of MSDC-0160, A Prototype mTOT Modulating Insulin Sensitizer, in Patients with Mild Alzheimer’s Disease

(2014) Raj Shah et al.   Current Alzheimer Research

Evidence for a Direct Effect of the NAD+Precursor Acipimox on Muscle Mitochondrial Function in Humans

(2014) Tineke van de Weijer et al.   DIABETES

The Efficacy and Safety of Imeglimin as Add-on Therapy in Patients With Type 2 Diabetes Inadequately Controlled With Sitagliptin Monotherapy

(2014) Pascale Fouqueray et al.   DIABETES CARE

The broadening spectrum of mitochondrial disease: Shifts in the diagnostic paradigm

(2013) Christina Liang et al.   BIOCHIMICA ET BIOPHYSICA ACTA-GENERAL SUBJECTS

Pioglitazone halts axonal degeneration in a mouse model of X-linked adrenoleukodystrophy

(2013) Laia Morató et al.   BRAIN

Persistence of the treatment effect of idebenone in Leber’s hereditary optic neuropathy

(2013) T. Klopstock et al.   BRAIN

Chronic Reduction of Plasma Free Fatty Acid Improves Mitochondrial Function and Whole-Body Insulin Sensitivity in Obese and Type 2 Diabetic Individuals

(2013) G. Daniele et al.   DIABETES

Reduction of non-esterified fatty acids improves insulin sensitivity and lowers oxidative stress, but fails to restore oxidative capacity in type 2 diabetes: a randomised clinical trial

(2013) Esther Phielix et al.   DIABETOLOGIA

Insulin sensitizers in 2013: new insights for the development of novel therapeutic agents to treat metabolic diseases

(2013) Jerry R Colca et al.   EXPERT OPINION ON INVESTIGATIONAL DRUGS

Structural basis for potent inhibitory activity of the antibiotic tigecycline during protein synthesis

(2013) L. Jenner et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Thiazolidinediones are acute, specific inhibitors of the mitochondrial pyruvate carrier

(2013) A. S. Divakaruni et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Mitochondrial Dynamics in Cardiovascular Health and Disease

(2012) Sang-Bing Ong et al.   ANTIOXIDANTS & REDOX SIGNALING

The Efficacy and Safety of Imeglimin as Add-on Therapy in Patients With Type 2 Diabetes Inadequately Controlled With Metformin Monotherapy

(2012) P. Fouqueray et al.   DIABETES CARE

Imeglimin, a novel glimin oral antidiabetic, exhibits a good efficacy and safety profile in type 2 diabetic patients

(2012) V. Pirags et al.   DIABETES OBESITY & METABOLISM

Mitochondria and cell signalling

(2012) Stephen W. G. Tait et al.   JOURNAL OF CELL SCIENCE

A novel Drp1 inhibitor diminishes aberrant mitochondrial fission and neurotoxicity

(2012) X. Qi et al.   JOURNAL OF CELL SCIENCE

Effects of Idebenone on Color Vision in Patients With Leber Hereditary Optic Neuropathy

(2012) Guenther Rudolph et al.   JOURNAL OF NEURO-OPHTHALMOLOGY

Features of Idebenone and Related Short-Chain Quinones that Rescue ATP Levels under Conditions of Impaired Mitochondrial Complex I

(2012) Michael Erb et al.   PLoS One

Mitochondrial dysfunction in pancreatic β cells

(2012) Sachin Supale et al.   TRENDS IN ENDOCRINOLOGY AND METABOLISM

Effects of long-term treatment with pioglitazone on cognition and glucose metabolism of PS1-KI, 3xTg-AD and wild-type mice

(2012) F Masciopinto et al.   Cell Death & Disease

The effects of idebenone on mitochondrial bioenergetics

(2011) Valentina Giorgio et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

Cysteamine restores glutathione redox status in cultured cystinotic proximal tubular epithelial cells

(2011) Martijn J. Wilmer et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

α-Tocotrienol quinone modulates oxidative stress response and the biochemistry of aging

(2011) William D. Shrader et al.   BIOORGANIC & MEDICINAL CHEMISTRY LETTERS

A randomized placebo-controlled trial of idebenone in Leber’s hereditary optic neuropathy

(2011) Thomas Klopstock et al.   BRAIN

Excessive Hepatic Mitochondrial TCA Cycle and Gluconeogenesis in Humans with Nonalcoholic Fatty Liver Disease

(2011) Nishanth E. Sunny et al.   Cell Metabolism

Phase I and pharmacokinetic study of COL-3 in patients with recurrent high-grade gliomas

(2011) Michelle A. Rudek et al.   JOURNAL OF NEURO-ONCOLOGY

Assessment of neurological efficacy of idebenone in pediatric patients with Friedreich's ataxia: data from a 6-month controlled study followed by a 12-month open-label extension study

(2011) Thomas Meier et al.   JOURNAL OF NEUROLOGY

Idebenone as a novel, therapeutic approach for Duchenne muscular dystrophy: Results from a 12 month, double-blind, randomized placebo-controlled trial

(2011) Gunnar M. Buyse et al.   NEUROMUSCULAR DISORDERS

Novel, Broad Spectrum Anticancer Agents Containing the Tricyclic 5:7:5-Fused Diimidazodiazepine Ring System

(2011) Atul Kondaskar et al.   ACS Medicinal Chemistry Letters

A Phase 3, Double-blind, Placebo-Controlled Trial of Idebenone in Friedreich Ataxia

(2010) David R. Lynch et al.   ARCHIVES OF NEUROLOGY

Metabolic control of mitochondrial biogenesis through the PGC-1 family regulatory network

(2010) Richard C. Scarpulla   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

Inhibiting Mitochondrial Fission Protects the Heart Against Ischemia/Reperfusion Injury

(2010) Sang-Bing Ong et al.   CIRCULATION

Pharmacokinetic evaluation of idebenone

(2010) Claudia Becker et al.   Expert Opinion on Drug Metabolism & Toxicology

Pioglitazone, Vitamin E, or Placebo for Nonalcoholic Steatohepatitis

(2010) Arun J. Sanyal et al.   NEW ENGLAND JOURNAL OF MEDICINE

Pioglitazone stimulates AMP-activated protein kinase signalling and increases the expression of genes involved in adiponectin signalling, mitochondrial function and fat oxidation in human skeletal muscle in vivo: a randomised trial

(2009) D. K. Coletta et al.   DIABETOLOGIA

Role of mitofusin 2 mutations in the physiopathology of Charcot–Marie–Tooth disease type 2A

(2009) Romain Cartoni et al.   EXPERIMENTAL NEUROLOGY

How mitochondria produce reactive oxygen species

(2008) Michael P. Murphy   BIOCHEMICAL JOURNAL

Chemical Inhibition of the Mitochondrial Division Dynamin Reveals Its Role in Bax/Bak-Dependent Mitochondrial Outer Membrane Permeabilization

(2008) Ann Cassidy-Stone et al.   DEVELOPMENTAL CELL

Randomized, Placebo-Controlled Trial of Pioglitazone in Nondiabetic Subjects With Nonalcoholic Steatohepatitis

(2008) Guruprasad P. Aithal et al.   GASTROENTEROLOGY