Mitochondrial iron and energetic dysfunction distinguish fibroblasts and induced neurons from pantothenate kinase-associated neurodegeneration patients

Neurobiology of Disease

Volumn 81, Issue , 2015, Pages 144-153

  Santambrogio, Paolo ^a   Dusi, Sabrina ^b   Guaraldo, Michela ^a,c   Rotundo, Luisa Ida ^a   Broccoli, Vania ^a   Garavaglia, Barbara ^b   Tiranti, Valeria ^b   Levi, Sonia ^a,c  

^a SAN RAFFAELE HOSPITAL   (Italy)

^b DEPARTMENT OF NEUROSURGERY   (Italy)

^c VITA SALUTE SAN RAFFAELE UNIVERSITY   (Italy)

Author keywords

Induced neurons; Iron metabolism; Mitochondria; NBIA; PANK2; Reactive oxygen species

Indexed keywords

ACONITATE HYDRATASE; ADENOSINE TRIPHOSPHATE; IRON; PANTOTHENATE KINASE; GLUTATHIONE; PHOSPHOTRANSFERASE; REACTIVE OXYGEN METABOLITE;

ADULT; ARTICLE; BRAIN MITOCHONDRION; BRAIN NERVE CELL; CELL FUNCTION; CELL LEVEL; CELL SHAPE; CONTROLLED STUDY; DISEASE ACTIVITY; DISORDERS OF MITOCHONDRIAL FUNCTIONS; ENZYME ACTIVITY; ENZYME ASSAY; HUMAN; MITOCHONDRIAL MEMBRANE POTENTIAL; MOLECULAR DYNAMICS; MOLECULAR PATHOLOGY; NEURODEGENERATION WITH BRAIN IRON ACCUMULATION; NEWBORN; NUCLEAR REPROGRAMMING; OXIDATIVE STRESS; PRIORITY JOURNAL; SKIN FIBROBLAST; ANALYSIS OF VARIANCE; CELL CULTURE; DEGENERATIVE DISEASE; ENERGY METABOLISM; ENZYMOLOGY; FIBROBLAST; GENETICS; LIP; METABOLISM; MITOCHONDRION; MUTATION; NERVE CELL; OXIDATION REDUCTION REACTION; PATHOLOGY; PHYSIOLOGY; ULTRASTRUCTURE;

ACONITATE HYDRATASE; ADENOSINE TRIPHOSPHATE; ADULT; ANALYSIS OF VARIANCE; CELLS, CULTURED; ENERGY METABOLISM; FIBROBLASTS; GLUTATHIONE; HUMANS; INFANT, NEWBORN; IRON; LIP; MEMBRANE POTENTIAL, MITOCHONDRIAL; MITOCHONDRIA; MUTATION; NEURODEGENERATIVE DISEASES; NEURONS; OXIDATION-REDUCTION; PHOSPHOTRANSFERASES (ALCOHOL GROUP ACCEPTOR); REACTIVE OXYGEN SPECIES;

EID: 84944911043     PISSN: 09699961     EISSN: 1095953X     Source Type: Journal    
DOI: 10.1016/j.nbd.2015.02.030     Document Type: Article

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