Glutathione: A redox signature in monitoring EPI-743 therapy in children with mitochondrial encephalomyopathies

Molecular Genetics and Metabolism

Volumn 109, Issue 2, 2013, Pages 208-214

  Pastore, Anna ^a   Petrillo, Sara ^a   Tozzi, Giulia ^a   Carrozzo, Rosalba ^a   Martinelli, Diego ^a   Dionisi Vici, Carlo ^a   Di Giovamberardino, Gianna ^a   Ceravolo, Ferdinando ^e   Klein, Matthew B ^b   Miller, Guy ^b,c   Enns, Gregory M ^d   Bertini, Enrico ^a   Piemonte, Fiorella ^a  

^a BAMBINO GESÙ CHILDREN S HOSPITAL   (Italy)

^b BioElectron Technology Corporation   (United States)

^c STANFORD UNIVERSITY   (United States)

^d LUCILE PACKARD CHILDREN S HOSPITAL   (United States)

^e UNIVERSITY MAGNA GRAECIA OF CATANZARO   (Italy)

Author keywords

EPI 743; Glutathione; Leigh syndrome; Mitochondrial encephalomyopathies; Oxidative stress

Indexed keywords

CARBONYL DERIVATIVE; GLUTATHIONE; GLUTATHIONE DISULFIDE; GLUTATHIONE PEROXIDASE; MALONALDEHYDE; SUPEROXIDE DISMUTASE; THIOL; VATIQUINONE;

ADOLESCENT; ARTICLE; BLOOD SAMPLING; CHILD; CLINICAL ARTICLE; CONTROLLED STUDY; ENZYME ACTIVITY; ERYTHROCYTE; FEMALE; FLUOROMETRY; HIGH PERFORMANCE LIQUID CHROMATOGRAPHY; HUMAN; HUMAN CELL; LEIGH DISEASE; LYMPHOCYTE; MALE; MITOCHONDRIAL ENCEPHALOMYOPATHY; OXIDATION REDUCTION REACTION; PRESCHOOL CHILD; PRIORITY JOURNAL; SCHOOL CHILD; SPECTROPHOTOMETRY;

ADOLESCENT; CASE-CONTROL STUDIES; CHILD; CHILD, PRESCHOOL; ERYTHROCYTES; GLUTATHIONE; GLUTATHIONE PEROXIDASE; HUMANS; INFANT; MALONDIALDEHYDE; MITOCHONDRIAL ENCEPHALOMYOPATHIES; OXIDATION-REDUCTION; SUPEROXIDE DISMUTASE; TREATMENT OUTCOME; UBIQUINONE;

EID: 84878490948     PISSN: 10967192     EISSN: 10967206     Source Type: Journal    
DOI: 10.1016/j.ymgme.2013.03.011     Document Type: Article

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