Vulnerability to oxidative stress in vitro in pathophysiology of mitochondrial short-chain Acyl-CoA dehydrogenase deficiency: Response to antioxidants

PLoS ONE

Volumn 6, Issue 4, 2011, Pages

  Zolkipli, Zarazuela ^a   Pedersen, Christina B ^b   Lamhonwah, Anne Marie ^a   Gregersen, Niels ^b   Tein, Ingrid ^a  

^a HOSPITAL FOR SICK CHILDREN   (Canada)

^b AARHUS UNIVERSITY HOSPITAL   (Denmark)

Author keywords

[No Author keywords available]

Indexed keywords

ACETYLCYSTEINE; ALPHA TOCOPHEROL; ANTIOXIDANT; ASCORBIC ACID; BEZAFIBRATE; CARNITINE PALMITOYLTRANSFERASE; CARNITINE PALMITOYLTRANSFERASE 2; ENOYL COENZYME A HYDRATASE; GLUCOSE; MANGANESE SUPEROXIDE DISMUTASE; MEDIUM CHAIN ACYL COENZYME A DEHYDROGENASE; MENADIONE; MITOCHONDRIAL PROTEIN; MITOCHONDRIAL TRIFUNCTIONAL PROTEIN; PEROXISOME PROLIFERATOR ACTIVATED RECEPTOR; UNCLASSIFIED DRUG; ACYL COENZYME A DEHYDROGENASE;

ADOLESCENT; ARTICLE; BUTYRYL COENZYME A DEHYDROGENASE DEFICIENCY; CELL DEATH; CELL VIABILITY; CONTROLLED STUDY; DISEASE EXACERBATION; DRUG EFFICACY; DRUG RESPONSE; ENZYME ACTIVITY; ENZYME DEFICIENCY; FATTY ACID OXIDATION; FIBROBLAST; GENOTYPE; GENOTYPE PHENOTYPE CORRELATION; HUMAN; HUMAN CELL; HYPERTHERMIA; HYPOGLYCEMIA; IN VITRO STUDY; NEUROTOXICITY; OXIDATIVE STRESS; PATHOPHYSIOLOGY; PROTEIN EXPRESSION; PROTEOMICS; RISK FACTOR; DISORDERS OF LIPID AND LIPOPROTEIN METABOLISM; DRUG EFFECT; METABOLISM; MITOCHONDRION; PATHOLOGY;

ACYL-COA DEHYDROGENASE; ADOLESCENT; ANTIOXIDANTS; FIBROBLASTS; HUMANS; LIPID METABOLISM, INBORN ERRORS; MITOCHONDRIA; OXIDATIVE STRESS; VITAMIN K 3;

EID: 79953662099     PISSN: None     EISSN: 19326203     Source Type: Journal    
DOI: 10.1371/journal.pone.0017534     Document Type: Article

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