Treatment of Mitochondrial Disorders

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

(2013) T. Klopstock et al.   BRAIN

First-in-class cardiolipin-protective compound as a therapeutic agent to restore mitochondrial bioenergetics

(2013) Hazel H Szeto   BRITISH JOURNAL OF PHARMACOLOGY

Targeting mitochondrial cardiolipin and the cytochromec/cardiolipin complex to promote electron transport and optimize mitochondrial ATP synthesis

(2013) A V Birk et al.   BRITISH JOURNAL OF PHARMACOLOGY

l-carnitine supplementation as a potential antioxidant therapy for inherited neurometabolic disorders

(2013) Graziela S. Ribas et al.   GENE

Reduction of Early Reperfusion Injury With the Mitochondria-Targeting Peptide Bendavia

(2013) David A. Brown et al.   JOURNAL OF CARDIOVASCULAR PHARMACOLOGY AND THERAPEUTICS

Mitochondrial targeted peptides attenuate residual myocardial damage after reversal of experimental renovascular hypertension

(2013) Alfonso Eirin et al.   JOURNAL OF HYPERTENSION

Practice patterns of mitochondrial disease physicians in North America. Part 2: treatment, care and management

(2013) Sumit Parikh et al.   MITOCHONDRION

Coenzyme Q10 deficiency in mitochondrial DNA depletion syndromes

(2013) Raquel Montero et al.   MITOCHONDRION

Practice patterns of mitochondrial disease physicians in North America. Part 1: Diagnostic and clinical challenges

(2013) Sumit Parikh et al.   MITOCHONDRION

Glutathione: A redox signature in monitoring EPI-743 therapy in children with mitochondrial encephalomyopathies

(2013) Anna Pastore et al.   MOLECULAR GENETICS AND METABOLISM

Review of Clinical Trials for Mitochondrial Disorders: 1997–2012

(2013) Douglas S. Kerr   Neurotherapeutics

Medical treatment with thiamine, coenzyme Q, vitamins E and C, and carnitine improved obstructive sleep apnea in an adult case of Leigh disease

(2013) Charalampos Mermigkis et al.   Sleep and Breathing

Leigh syndrome associated with mitochondrial complex I deficiency due to novel mutations In NDUFV1 and NDUFS2

(2012) Samantha E. Marin et al.   GENE

Update on clinical aspects and treatment of selected vitamin-responsive disorders II (riboflavin and CoQ10)

(2012) Rita Horvath   JOURNAL OF INHERITED METABOLIC DISEASE

EPI-743 reverses the progression of the pediatric mitochondrial disease—Genetically defined Leigh Syndrome

(2012) Diego Martinelli et al.   MOLECULAR GENETICS AND METABOLISM

Brain uptake of Tc99m-HMPAO correlates with clinical response to the novel redox modulating agent EPI-743 in patients with mitochondrial disease

(2012) Francis G. Blankenberg et al.   MOLECULAR GENETICS AND METABOLISM

Reduction of Ischemia/Reperfusion Injury With Bendavia, a Mitochondria‐Targeting Cytoprotective Peptide

(2012) Robert A. Kloner et al.   Journal of the American Heart Association

Cocoa and Chocolate in Human Health and Disease

(2011) David L. Katz et al.   ANTIOXIDANTS & REDOX SIGNALING

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

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

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

(-)-Epicatechin enhances fatigue resistance and oxidative capacity in mouse muscle

(2011) Leonardo Nogueira et al.   JOURNAL OF PHYSIOLOGY-LONDON

Why are there no proven therapies for genetic mitochondrial diseases?

(2011) Peter W. Stacpoole   MITOCHONDRION

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

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

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

(2010) Dao-Fu Dai et al.   AGING CELL

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

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

Coenzyme Q and mitochondrial disease

(2010) Catarina M. Quinzii et al.   Developmental Disabilities Research Reviews

Metabolic manipulators: a well founded strategy to combat mitochondrial dysfunction

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

A randomized trial of coenzyme Q10in mitochondrial disorders

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

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

A modern approach to the treatment of mitochondrial disease

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

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

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

The mitochondrial cocktail: Rationale for combined nutraceutical therapy in mitochondrial cytopathies☆

(2008) M.A. Tarnopolsky   ADVANCED DRUG DELIVERY REVIEWS

Short-term effects of coenzyme Q10 in progressive supranuclear palsy: A randomized, placebo-controlled trial

(2008) Maria Stamelou et al.   MOVEMENT DISORDERS