Partial complex I deficiency due to the CNS conditional ablation of Ndufa5 results in a mild chronic encephalopathy but no increase in oxidative damage

Which way does the citric acid cycle turn during hypoxia? The critical role of α-ketoglutarate dehydrogenase complex

(2013) Christos Chinopoulos   JOURNAL OF NEUROSCIENCE RESEARCH

Crystal structure of the entire respiratory complex I

(2013) Rozbeh Baradaran et al.   NATURE

Mitochondrial DNA damage in a mouse model of Alzheimer's disease decreases amyloid beta plaque formation

(2013) Milena Pinto et al.   NEUROBIOLOGY OF AGING

A defect in the mitochondrial complex III, but not complex IV, triggers early ROS-dependent damage in defined brain regions

(2012) Francisca Diaz et al.   HUMAN MOLECULAR GENETICS

Proteomic and Metabolomic Analyses of Mitochondrial Complex I-deficient Mouse Model Generated by Spontaneous B2 Short Interspersed Nuclear Element (SINE) Insertion into NADH Dehydrogenase (Ubiquinone) Fe-S Protein 4 (Ndufs4) Gene

(2012) Dillon W. Leong et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Fatal breathing dysfunction in a mouse model of Leigh syndrome

(2012) Albert Quintana et al.   JOURNAL OF CLINICAL INVESTIGATION

Mouse mtDNA mutant model of Leber hereditary optic neuropathy

(2012) C. S. Lin et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Tissue-specific splicing of an Ndufs6 gene-trap insertion generates a mitochondrial complex I deficiency-specific cardiomyopathy

(2012) B.-X. Ke et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Understanding mitochondrial complex I assembly in health and disease

(2011) Masakazu Mimaki et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

Assembly factors as a new class of disease genes for mitochondrial complex I deficiency: cause, pathology and treatment options

(2011) Jessica Nouws et al.   BRAIN

Mitochondrial complex I deficiency of nuclear origin

(2011) Hélène Pagniez-Mammeri et al.   MOLECULAR GENETICS AND METABOLISM

Mitochondrial complex I deficiency of nuclear origin

(2011) Hélène Pagniez-Mammeri et al.   MOLECULAR GENETICS AND METABOLISM

2-Deoxy-D-Glucose Treatment Induces Ketogenesis, Sustains Mitochondrial Function, and Reduces Pathology in Female Mouse Model of Alzheimer's Disease

(2011) Jia Yao et al.   PLoS One

Mitochondrial disorders caused by mutations in respiratory chain assembly factors

(2011) Francisca Diaz et al.   Seminars in Fetal & Neonatal Medicine

The electron transfer flavoprotein: Ubiquinone oxidoreductases

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

Ketogenic diet slows down mitochondrial myopathy progression in mice

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

Mouse models for nuclear DNA-encoded mitochondrial complex I deficiency

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

Mitochondrial DNA Mutations Induce Mitochondrial Dysfunction, Apoptosis and Sarcopenia in Skeletal Muscle of Mitochondrial DNA Mutator Mice

(2010) Asimina Hiona et al.   PLoS One

Complex I deficiency due to loss of Ndufs4 in the brain results in progressive encephalopathy resembling Leigh syndrome

(2010) A. Quintana et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

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

(2010) C. Hunte et al.   SCIENCE

Mammalian Mitochondrial Complex I: Biogenesis, Regulation, and Reactive Oxygen Species Generation

(2009) Werner J.H. Koopman et al.   ANTIOXIDANTS & REDOX SIGNALING

Mitochondrial complex I deficiency: from organelle dysfunction to clinical disease

(2009) F. Distelmaier et al.   BRAIN

RETRACTED: mTERF2 Regulates Oxidative Phosphorylation by Modulating mtDNA Transcription

(2009) Tina Wenz et al.   Cell Metabolism

NDUFS4: Creation of a mouse model mimicking a Complex I disorder

(2009) Christopher A. Ingraham et al.   MITOCHONDRION

Mitochondrial Disorders in the Nervous System

(2008) Salvatore DiMauro et al.   Annual Review of Neuroscience

How mitochondria produce reactive oxygen species

(2008) Michael P. Murphy   BIOCHEMICAL JOURNAL

Mice with Mitochondrial Complex I Deficiency Develop a Fatal Encephalomyopathy

(2008) Shane E. Kruse et al.   Cell Metabolism

The Biology of Cancer: Metabolic Reprogramming Fuels Cell Growth and Proliferation

(2008) Ralph J. DeBerardinis et al.   Cell Metabolism

The ketogenic diet: Uses in epilepsy and other neurologic illnesses

(2008) Kristin W. Barañano et al.   Current Treatment Options in Neurology

Electrophoresis techniques to investigate defects in oxidative phosphorylation

(2008) Maria Antonietta Calvaruso et al.   METHODS

Respiratory Active Mitochondrial Supercomplexes

(2008) Rebeca Acín-Pérez et al.   MOLECULAR CELL

The mitochondrial impairment, oxidative stress and neurodegeneration connection: reality or just an attractive hypothesis?

(2008) Hirokazu Fukui et al.   TRENDS IN NEUROSCIENCES