iPSC-derived neuronal models of PANK2-associated neurodegeneration reveal mitochondrial dysfunction contributing to early disease

PLoS ONE

Volumn 12, Issue 9, 2017, Pages

  Arber, Charles ^a   Angelova, Plamena R ^a   Wiethoff, Sarah ^a   Tsuchiya, Yugo ^b   Mazzacuva, Francesca ^c   Preza, Elisavet ^a   Bhatia, Kailash P ^a   Mills, Kevin ^c   Gout, Ivan ^b   Abramov, Andrey Y ^a   Hardy, John ^a   Duce, James A ^d   Houlden, Henry ^a   Wray, Selina ^a  

^a UNIVERSITY COLLEGE LONDON   (United Kingdom)

^b UNIVERSITY COLLEGE LONDON   (United Kingdom)

^c UNIVERSITY COLLEGE LONDON   (United Kingdom)

^d UNIVERSITY OF LEEDS   (United Kingdom)

Author keywords

[No Author keywords available]

Indexed keywords

5,10 METHYLENETETRAHYDROFOLATE REDUCTASE (FADH2); COENZYME A; PANTOTHENATE KINASE 2; REACTIVE OXYGEN METABOLITE; REDUCED NICOTINAMIDE ADENINE DINUCLEOTIDE; ACETYL COENZYME A; IRON; NICOTINAMIDE ADENINE DINUCLEOTIDE; PANTOTHENATE KINASE; PANTOTHENIC ACID; PHOSPHOTRANSFERASE;

ARTICLE; BRAIN CELL CULTURE; CELL DIFFERENTIATION; CONTROLLED STUDY; FIBROBLAST; GENE MUTATION; HUMAN; HUMAN CELL; INDUCED PLURIPOTENT STEM CELL; IRON CHELATION; LIPID PEROXIDATION; MITOCHONDRIAL DYSFUNCTION; MITOCHONDRIAL MEMBRANE POTENTIAL; MITOCHONDRION; NERVE CELL; NEURODEGENERATION WITH BRAIN IRON ACCUMULATION; NUCLEAR REPROGRAMMING; OXIDATIVE PHOSPHORYLATION; PROTEIN EXPRESSION; ADOLESCENT; BIOPSY; BRAIN; CHEMISTRY; CHILD; CYTOLOGY; DISORDERS OF MITOCHONDRIAL FUNCTIONS; FEMALE; GENETICS; KARYOTYPING; MALE; METABOLISM; MUTATION; PATHOLOGY; PATHOPHYSIOLOGY; PHENOTYPE; PLASMID;

ACETYL COENZYME A; ADOLESCENT; BIOPSY; BRAIN; CELL DIFFERENTIATION; CHILD; COENZYME A; FEMALE; FIBROBLASTS; HUMANS; INDUCED PLURIPOTENT STEM CELLS; IRON; KARYOTYPING; LIPID PEROXIDATION; MALE; MEMBRANE POTENTIAL, MITOCHONDRIAL; MITOCHONDRIA; MITOCHONDRIAL DISEASES; MUTATION; NAD; NEURONS; PANTOTHENATE KINASE-ASSOCIATED NEURODEGENERATION; PANTOTHENIC ACID; PHENOTYPE; PHOSPHOTRANSFERASES (ALCOHOL GROUP ACCEPTOR); PLASMIDS; REACTIVE OXYGEN SPECIES;

EID: 85029849263     PISSN: None     EISSN: 19326203     Source Type: Journal    
DOI: 10.1371/journal.pone.0184104     Document Type: Article

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