Drugs and mitochondrial diseases: 40 queries and answers

Neuroprotective Effects of Tetracyclines: Molecular Targets, Animal Models and Human Disease

(2012) D. Orsucci et al.   CNS & Neurological Disorders-Drug Targets

Tetracycline treatment in patients with progressive external ophthalmoplegia

(2011) M. Mancuso et al.   ACTA NEUROLOGICA SCANDINAVICA

Lipoic Acid Synthetase Deficiency Causes Neonatal-Onset Epilepsy, Defective Mitochondrial Energy Metabolism, and Glycine Elevation

(2011) Johannes A. Mayr et al.   AMERICAN JOURNAL OF HUMAN GENETICS

The effects of idebenone on mitochondrial bioenergetics

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

Idebenone Treatment In Leber's Hereditary Optic Neuropathy

(2011) V. Carelli et al.   BRAIN

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

(2011) Thomas Klopstock et al.   BRAIN

Beneficial effect of pyruvate therapy on Leigh syndrome due to a novel mutation in PDH E1α gene

(2011) Yasutoshi Koga et al.   BRAIN & DEVELOPMENT

Malignant hyperthermia: pharmacology of triggering

(2011) P.M. Hopkins   BRITISH JOURNAL OF ANAESTHESIA

In Vivo Correction of COX Deficiency by Activation of the AMPK/PGC-1α Axis

(2011) Carlo Viscomi et al.   Cell Metabolism

Cellular and molecular mechanisms of metformin: an overview

(2011) Benoit Viollet et al.   CLINICAL SCIENCE

Targeting Mitochondrial Dysfunction and Neurodegeneration by Means of Coenzyme Q10 and its Analogues

(2011) D. Orsucci et al.   CURRENT MEDICINAL CHEMISTRY

Update on Toxic Myopathies

(2011) F. L. Mastaglia et al.   Current Neurology and Neuroscience Reports

Transmitochondrial Mice as Models for Mitochondrial DNA-Based Diseases

(2011) Kazuto NAKADA et al.   EXPERIMENTAL ANIMALS

Secondary coenzyme Q10 deficiency triggers mitochondria degradation by mitophagy in MELAS fibroblasts

(2011) David Cotán et al.   FASEB JOURNAL

adPEO mutations in ANT1 impair ADP-ATP translocation in muscle mitochondria

(2011) H. Kawamata et al.   HUMAN MOLECULAR GENETICS

Effect of bezafibrate treatment on late-onset mitochondrial myopathy in mice

(2011) Shuichi Yatsuga et al.   HUMAN MOLECULAR GENETICS

Loss of a Conserved Tyrosine Residue of CytochromebInduces Reactive Oxygen Species Production by Cytochromebc1

(2011) Dong-Woo Lee et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Rapid-onset, linezolid-induced lactic acidosis in MELAS

(2011) Thomas E. Cope et al.   MITOCHONDRION

Initial experience in the treatment of inherited mitochondrial disease with EPI-743

(2011) Gregory M. Enns et al.   MOLECULAR GENETICS AND METABOLISM

Metabolic acidosis with topiramate and zonisamide

(2011) Nasir S. Mirza et al.   Pharmacogenetics and Genomics

The Effect of OPA1 on Mitochondrial Ca2+ Signaling

(2011) László Fülöp 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

A yeast-based assay identifies drugs active against human mitochondrial disorders

(2011) E. Couplan et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Mutation analysis of mitochondrial 12S rRNA gene in Polish patients with non-syndromic and aminoglycoside-induced hearing loss

(2010) Małgorzata Rydzanicz et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

Bezafibrate induced increase in mitochondrial electron transport chain complex IV activity in human astrocytoma cells: Implications for mitochondrial cytopathies and neurodegenerative diseases

(2010) Nicola Ioannou et al.   BIOFACTORS

Oestrogens ameliorate mitochondrial dysfunction in Leber’s hereditary optic neuropathy

(2010) Carla Giordano et al.   BRAIN

Riboflavin-responsive oxidative phosphorylation complex I deficiency caused by defective ACAD9: new function for an old gene

(2010) Mike Gerards et al.   BRAIN

Acetaminophen reduces mitochondrial dysfunction during early cerebral postischemic reperfusion in rats

(2010) Sunanda S. Baliga et al.   BRAIN RESEARCH

Understanding lactic acidosis in paracetamol (acetaminophen) poisoning

(2010) Anoop D. Shah et al.   BRITISH JOURNAL OF CLINICAL PHARMACOLOGY

Treatment of central nervous system manifestations in mitochondrial disorders

(2010) J. Finsterer   EUROPEAN JOURNAL OF NEUROLOGY

Therapeutic use of coenzyme Q10and coenzyme Q10-related compounds and formulations

(2010) Jose M Villalba et al.   EXPERT OPINION ON INVESTIGATIONAL DRUGS

Ketogenic diet slows down mitochondrial myopathy progression in mice

(2010) Sofia Ahola-Erkkilä et al.   HUMAN MOLECULAR GENETICS

Metabolic manipulators: a well founded strategy to combat mitochondrial dysfunction

(2010) Saskia Koene et al.   JOURNAL OF INHERITED METABOLIC DISEASE

Magnesium treatment for patients with refractory status epilepticus due to POLG1-mutations

(2010) Nora A. Visser et al.   JOURNAL OF NEUROLOGY

Mitochondrial 12S rRNA variants in 1642 Han Chinese pediatric subjects with aminoglycoside-induced and nonsyndromic hearing loss

(2010) Jianxin Lu et al.   MITOCHONDRION

A randomized trial of coenzyme Q10in mitochondrial disorders

(2010) Elisa I. Glover et al.   MUSCLE & NERVE

Combined treatment with oral metronidazole and N-acetylcysteine is effective in ethylmalonic encephalopathy

(2010) Carlo Viscomi et al.   NATURE MEDICINE

Metabolic acidosis: pathophysiology, diagnosis and management

(2010) Jeffrey A. Kraut et al.   Nature Reviews Nephrology

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

Safety of Vitamins and Minerals: Controversies and Perspective

(2010) Madhu G. Soni et al.   TOXICOLOGICAL SCIENCES

Interaction of Aminoglycosides with Human Mitochondrial 12S rRNA Carrying the Deafness-Associated Mutation

(2009) Y. Qian et al.   ANTIMICROBIAL AGENTS AND CHEMOTHERAPY

Human CoQ10deficiencies

(2009) C. M. Quinzii et al.   BIOFACTORS

A modern approach to the treatment of mitochondrial disease

(2009) Sumit Parikh et al.   Current Treatment Options in Neurology

Drugs interfering with mitochondrial disorders

(2009) Josef Finsterer et al.   DRUG AND CHEMICAL TOXICOLOGY

Management of mitochondrial stroke-like-episodes

(2009) J. Finsterer   EUROPEAN JOURNAL OF NEUROLOGY

Chronic impact of topiramate on acid-base balance and potassium in childhood

(2009) Eva A. Belotti et al.   EUROPEAN JOURNAL OF PAEDIATRIC NEUROLOGY

Impairment of mitochondrial function by minocycline

(2009) Kathleen Kupsch et al.   FEBS Journal

Long-term therapy with clevudine for chronic hepatitis B can be associated with myopathy characterized by depletion of mitochondrial DNA

(2009) Jung Im Seok et al.   HEPATOLOGY

The interactions of some commonly consumed drugs with mitochondrial adaptations to exercise

(2009) Matthew M. Robinson et al.   JOURNAL OF APPLIED PHYSIOLOGY

Severe Non-Anion Gap Metabolic Acidosis Induced by Topiramate: A Case Report

(2009) Joseph R. Shiber   JOURNAL OF EMERGENCY MEDICINE

Oxidative stress biomarkers in mitochondrial myopathies, basally and after cysteine donor supplementation

(2009) Michelangelo Mancuso et al.   JOURNAL OF NEUROLOGY

Antibiotic effects on mitochondrial translation and in patients with mitochondrial translational defects

(2009) Christie N. Jones et al.   MITOCHONDRION

Treatment of mitochondrial electron transport chain disorders: A review of clinical trials over the past decade

(2009) Douglas S. Kerr   MOLECULAR GENETICS AND METABOLISM

Mitochondrial gene replacement in primate offspring and embryonic stem cells

(2009) Masahito Tachibana et al.   NATURE

OPHTHALMOPLEGIA AND PTOSIS: MITOCHONDRIAL TOXICITY IN PATIENTS RECEIVING HIV THERAPY

(2009) G. Pfeffer et al.   NEUROLOGY

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

(2009) Sabrina Sacconi et al.   NEUROMUSCULAR DISORDERS

Electron transfer mediators and other metabolites and cofactors in the treatment of mitochondrial dysfunction

(2009) Daniele Orsucci et al.   NUTRITION REVIEWS

Inherited disorders affecting mitochondrial function are associated with glutathione deficiency and hypocitrullinemia

(2009) K. R. Atkuri et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Coenzyme Q10 and alpha-tocopherol protect against amitriptyline toxicity

(2009) Mario D. Cordero et al.   TOXICOLOGY AND APPLIED PHARMACOLOGY

Clinical implications of mitochondrial disease☆

(2008) Stanley Muravchick   ADVANCED DRUG DELIVERY REVIEWS

Metabolic acidosis in the critically ill: Part 2. Causes and treatment

(2008) C. G. Morris et al.   ANAESTHESIA

Intestinal pseudo-obstruction in a patient with mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) associated with phenytoin therapy

(2008) Tomohiro Chiyonobu et al.   BRAIN & DEVELOPMENT

Water-soluble Coenzyme Q10 formulation (Q-ter) promotes outer hair cell survival in a guinea pig model of noise induced hearing loss (NIHL)

(2008) Anna Rita Fetoni et al.   BRAIN RESEARCH

RETRACTED: Activation of the PPAR/PGC-1α Pathway Prevents a Bioenergetic Deficit and Effectively Improves a Mitochondrial Myopathy Phenotype

(2008) Tina Wenz et al.   Cell Metabolism

Severe lactic acidosis secondary to minocycline in a teenager with infectious mononucleosis and mitochondrial myopathy

(2008) Richard M. Zoraster et al.   CLINICAL NEUROLOGY AND NEUROSURGERY

Role of Na+/L-carnitine Transporter (OCTN2) in Renal Handling of Pivaloylcarnitine and Valproylcarnitine Formed during Pivalic Acid-containing Prodrugs and Valproic Acid Treatment

(2008) Shuichi Ohnishi et al.   Drug Metabolism and Pharmacokinetics

Electrophysiology and Pharmacology of Striatal Neuronal Dysfunction Induced by Mitochondrial Complex I Inhibition

(2008) C. Costa et al.   JOURNAL OF NEUROSCIENCE

Myotoxicity of Lipid-Lowering Agents in a Teenager With MELAS Mutation

(2008) Stacey K.H. Tay et al.   PEDIATRIC NEUROLOGY

Genetic analysis of interactions with eukaryotic rRNA identify the mitoribosome as target in aminoglycoside ototoxicity

(2008) S. N. Hobbie et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Prevalence of mitochondrial DNA disease in adults

(2007) Andrew M. Schaefer et al.   ANNALS OF NEUROLOGY