Neuronal and astrocyte dysfunction diverges from embryonic fibroblasts in theNdufs4fky/fkymouse

Mitochondria: Mitochondrial OXPHOS (dys) function ex vivo – The use of primary fibroblasts

(2014) Ann Saada   INTERNATIONAL JOURNAL OF BIOCHEMISTRY & CELL BIOLOGY

Modelling biochemical features of mitochondrial neuropathology

(2013) Matthew J. Bird et al.   BIOCHIMICA ET BIOPHYSICA ACTA-GENERAL SUBJECTS

Unsolved issues related to human mitochondrial diseases

(2013) Anne Lombès et al.   BIOCHIMIE

A guide to diagnosis and treatment of Leigh syndrome

(2013) F. Baertling et al.   JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY

Mitochondrial metabolism of reactive oxygen species

(2013) Paola Venditti et al.   MITOCHONDRION

Astrocytic energetics during excitatory neurotransmission: What are contributions of glutamate oxidation and glycolysis?

(2013) Gerald A. Dienel   NEUROCHEMISTRY INTERNATIONAL

Sugar for the brain: the role of glucose in physiological and pathological brain function

(2013) Philipp Mergenthaler et al.   TRENDS IN NEUROSCIENCES

Unraveling the complex metabolic nature of astrocytes

(2013) Anne-Karine Bouzier-Sore et al.   Frontiers in Cellular Neuroscience

Metabolic consequences of NDUFS4 gene deletion in immortalized mouse embryonic fibroblasts

(2012) Federica Valsecchi et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

A constant and similar assembly defect of mitochondrial respiratory chain complex I allows rapid identification of NDUFS4 mutations in patients with Leigh syndrome

(2012) Z. Assouline et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

OXPHOS mutations and neurodegeneration

(2012) Werner J H Koopman et al.   EMBO JOURNAL

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

Primary fibroblasts of NDUFS4−/− mice display increased ROS levels and aberrant mitochondrial morphology

(2012) Federica Valsecchi et al.   MITOCHONDRION

Mitochondria: master regulators of danger signalling

(2012) Lorenzo Galluzzi et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

Diagnosis and treatment of mitochondrial myopathies

(2011) Gerald Pfeffer et al.   ANNALS OF MEDICINE

Mitochondrial complex III stabilizes complex I in the absence of NDUFS4 to provide partial activity

(2011) M. A. Calvaruso et al.   HUMAN MOLECULAR GENETICS

Astrocytes and Microglia: Responses to Neuropathological Conditions

(2011) Sarika Singh et al.   INTERNATIONAL JOURNAL OF NEUROSCIENCE

Superoxide Is Produced by the Reduced Flavin in Mitochondrial Complex I

(2011) Kenneth R. Pryde et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Loss of mitochondrial complex I activity potentiates dopamine neuron death induced by microtubule dysfunction in a Parkinson’s disease model

(2011) Won-Seok Choi et al.   JOURNAL OF CELL BIOLOGY

Mechanism of neurodegeneration of neurons with mitochondrial DNA mutations

(2010) A. Y. Abramov et al.   BRAIN

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

Specification and Morphogenesis of Astrocytes

(2010) M. R. Freeman   SCIENCE

Mitochondria as ATP consumers in cellular pathology

(2009) Christos Chinopoulos et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Mitochondrial complex I deficiency: from organelle dysfunction to clinical disease

(2009) F. Distelmaier et al.   BRAIN

The pathophysiology of mitochondrial disease as modeled in the mouse

(2009) D. C. Wallace et al.   GENES & DEVELOPMENT

Glycolysis: a bioenergetic or a survival pathway?

(2009) Juan P. Bolaños et al.   TRENDS IN BIOCHEMICAL SCIENCES

Mitochondrial Disorders in the Nervous System

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

Regulation of Mitochondrial Structure and Function by the F1Fo-ATPase Inhibitor Protein, IF1

(2008) Michelangelo Campanella et al.   Cell Metabolism

Mice with Mitochondrial Complex I Deficiency Develop a Fatal Encephalomyopathy

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

Glucose metabolism inhibits apoptosis in neurons and cancer cells by redox inactivation of cytochrome c

(2008) Allyson E. Vaughn et al.   NATURE CELL BIOLOGY

The Mystery and Magic of Glia: A Perspective on Their Roles in Health and Disease

(2008) Ben A. Barres   NEURON

Mitochondrial complex I inhibition is not required for dopaminergic neuron death induced by rotenone, MPP+, or paraquat

(2008) W.-S. Choi et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA