Complex I protein NDUFS2 is vital for growth, ROS generation, membrane integrity, apoptosis, and mitochondrial energetics

Ndufs2, A Core Subunit of Mitochondrial Complex I, Is Essential for Acute Oxygen-Sensing and Hypoxic Pulmonary Vasoconstriction

(2019) Kimberly J Dunham-Snary et al.   CIRCULATION RESEARCH

Mitochondrial Respiration and H2O2 Emission in Saponin-permeabilized Murine Diaphragm Fibers: Optimization of Fiber Separation and Comparison to Limb Muscle

(2019) Dongwoo Hahn et al.   AMERICAN JOURNAL OF PHYSIOLOGY-CELL PHYSIOLOGY

Mitochondrial Complex I Function Is Essential for Neural Stem/Progenitor Cells Proliferation and Differentiation

(2019) Daniel Cabello-Rivera et al.   Frontiers in Neuroscience

Influence of Pulsed Electric Fields and Mitochondria-Cytoskeleton Interactions on Cell Respiration

(2018) Ishan Goswami et al.   BIOPHYSICAL JOURNAL

Exercise-induced protection against reperfusion arrhythmia involves stabilization of mitochondrial energetics

(2016) Rick J. Alleman et al.   AMERICAN JOURNAL OF PHYSIOLOGY-HEART AND CIRCULATORY PHYSIOLOGY

Compound heterozygosity for severe and hypomorphic NDUFS2 mutations cause non-syndromic LHON-like optic neuropathy

(2016) Sylvie Gerber et al.   JOURNAL OF MEDICAL GENETICS

Accessory subunits are integral for assembly and function of human mitochondrial complex I

(2016) David A. Stroud et al.   NATURE

Idebenone reduces respiratory complications in patients with Duchenne muscular dystrophy

(2016) Craig M. McDonald et al.   NEUROMUSCULAR DISORDERS

Treatment effect of idebenone on inspiratory function in patients with Duchenne muscular dystrophy

(2016) Gunnar M. Buyse et al.   PEDIATRIC PULMONOLOGY

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

Complex I dysfunction underlies the glycolytic switch in pulmonary hypertensive smooth muscle cells

(2015) Ruslan Rafikov et al.   Redox Biology

Contemporary Treatment of Amyloid Heart Disease

(2014) Tomas Palecek et al.   CURRENT PHARMACEUTICAL DESIGN

Therapeutic approaches for the treatment of Friedreich’s ataxia

(2014) Cassandra J Strawser et al.   Expert Review of Neurotherapeutics

The antioxidant idebenone fails to prevent or attenuate chronic experimental autoimmune encephalomyelitis in the mouse

(2013) Sebastian M. Fiebiger et al.   JOURNAL OF NEUROIMMUNOLOGY

Crystal structure of the entire respiratory complex I

(2013) Rozbeh Baradaran et al.   NATURE

Genome engineering using the CRISPR-Cas9 system

(2013) F Ann Ran et al.   Nature Protocols

Molecular diagnosis in mitochondrial complex I deficiency using exome sequencing

(2012) Tobias B Haack et al.   JOURNAL OF MEDICAL GENETICS

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

Idebenone Protects against Retinal Damage and Loss of Vision in a Mouse Model of Leber’s Hereditary Optic Neuropathy

(2012) Fabrice D. Heitz et al.   PLoS One

Mutation screening of 75 candidate genes in 152 complex I deficiency cases identifies pathogenic variants in 16 genes includingNDUFB9

(2011) Tobias B Haack et al.   JOURNAL OF MEDICAL GENETICS

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

NQO1-Dependent Redox Cycling of Idebenone: Effects on Cellular Redox Potential and Energy Levels

(2011) Roman H. Haefeli et al.   PLoS One

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

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

The p.M292T NDUFS2 mutation causes complex I-deficient Leigh syndrome in multiple families

(2010) Helen A. L. Tuppen et al.   BRAIN

The sites and topology of mitochondrial superoxide production

(2010) Martin D. Brand   EXPERIMENTAL GERONTOLOGY

The architecture of respiratory complex I

(2010) Rouslan G. Efremov et al.   NATURE

Functional Modules and Structural Basis of Conformational Coupling in Mitochondrial Complex I

(2010) C. Hunte et al.   SCIENCE

Mitochondria and reactive oxygen species

(2009) Alicia J. Kowaltowski et al.   FREE RADICAL BIOLOGY AND MEDICINE