Pathogenic VCP Mutations Induce Mitochondrial Uncoupling and Reduced ATP Levels

Neuron

Volumn 78, Issue 1, 2013, Pages 57-64

  Bartolome, Fernando ^a   Wu, Hsiu Chuan ^a   Burchell, Victoria S ^a   Preza, Elisavet ^a   Wray, Selina ^a   Mahoney, Colin J ^a   Fox, Nick C ^a   Calvo, Andrea ^c   Canosa, Antonio ^c   Moglia, Cristina ^c   Mandrioli, Jessica ^d   Chiò, Adriano ^c   Orrell, Richard W ^a   Houlden, Henry ^a   Hardy, John ^a,b   Abramov, Andrey Y ^a   Plun Favreau, Helene ^a  

^a UNIVERSITY COLLEGE LONDON   (United Kingdom)

^b UNIVERSITY COLLEGE LONDON   (United Kingdom)

^c UNIVERSITY OF TURIN   (Italy)

^d UNIVERSITY OF MODENA AND REGGIO EMILIA   (Italy)

Author keywords

[No Author keywords available]

Indexed keywords

ADENOSINE TRIPHOSPHATE; FLAVINE ADENINE NUCLEOTIDE; REDUCED NICOTINAMIDE ADENINE DINUCLEOTIDE; SMALL INTERFERING RNA; VALOSIN CONTAINING PROTEIN;

ANIMAL CELL; APOPTOSIS; ARTICLE; CELL ENERGY; CELL MEMBRANE DEPOLARIZATION; CONTROLLED STUDY; DISEASE ASSOCIATION; ENERGY YIELD; FIBROBLAST; FRONTOTEMPORAL DEMENTIA; GENE MUTATION; GENE OVEREXPRESSION; GENE SILENCING; GENETIC TRANSFECTION; GLYCOLYSIS; HUMAN; HUMAN CELL; INCLUSION BODY MYOPATHY; INTRACELLULAR SIGNALING; LIPID PEROXIDATION; MITOCHONDRIAL MEMBRANE POTENTIAL; MITOCHONDRIAL RESPIRATION; MOLECULAR DYNAMICS; MOLECULAR PATHOLOGY; MOUSE; MYOPATHY; NEUROTOXICITY; NONHUMAN; OXIDATION REDUCTION STATE; OXIDATIVE PHOSPHORYLATION UNCOUPLING; OXYGEN CONSUMPTION; PAGET BONE DISEASE; PRIORITY JOURNAL; PROTEIN DEFICIENCY; RESPIRATORY CHAIN; VALOSIN CONTAINING PROTEIN GENE;

ADENOSINE TRIPHOSPHATASES; ADENOSINE TRIPHOSPHATE; ADULT; AGED; ANALYSIS OF VARIANCE; ANIMALS; ANIMALS, NEWBORN; CASE-CONTROL STUDIES; CELL CYCLE PROTEINS; CELLS, CULTURED; CEREBRAL CORTEX; FAMILY HEALTH; FEMALE; FIBROBLASTS; FRONTOTEMPORAL DEMENTIA; HUMANS; LIPID PEROXIDATION; LUMINESCENT PROTEINS; MAGNESIUM; MALE; MEMBRANE POTENTIAL, MITOCHONDRIAL; MICE; MICE, INBRED C57BL; MIDDLE AGED; MITOCHONDRIA; MUSCULAR DYSTROPHIES, LIMB-GIRDLE; MUTATION; MYOSITIS, INCLUSION BODY; NAD; NEUROBLASTOMA; NEURONS; OSTEITIS DEFORMANS; OXYGEN CONSUMPTION; RNA, SMALL INTERFERING; TRANSFECTION;

EID: 84876079328     PISSN: 08966273     EISSN: 10974199     Source Type: Journal    
DOI: 10.1016/j.neuron.2013.02.028     Document Type: Article

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