Multiple OXPHOS deficiency in the liver, kidney, heart, and skeletal muscle of patients with methylmalonic aciduria and propionic aciduria

Pediatric Research

Volumn 66, Issue 1, 2009, Pages 91-95

  De Keyzer, Yves ^a,b   Valayannopoulos, Vassili ^a   Benoist, Jean François ^c   Batteux, Frédéric ^d   Lacaille, Florence ^a   Hubert, Laurence ^b   Chrétien, Dominique ^b   Chadefeaux Vekemans, Bernadette ^a   Niaudet, Patrick ^a   Touati, Guy ^a   Munnich, Arnold ^b   De Lonlay, Pascale ^a,b  

^a HÔPITAL NECKER ENFANTS MALADES   (France)

^b INSERM   (France)

^c HÔPITAL ROBERT DEBRÉ   (France)

^d UNIVERSITÉ PARIS DESCARTES   (France)

Author keywords

[No Author keywords available]

Indexed keywords

AMOXICILLIN; BETA ADRENERGIC RECEPTOR BLOCKING AGENT; CARNITINE; CYANOCOBALAMIN; DIGOXIN; DIPEPTIDYL CARBOXYPEPTIDASE INHIBITOR; DIURETIC AGENT; METRONIDAZOLE; REACTIVE OXYGEN METABOLITE; SULFAMETHOXAZOLE; TRICARBOXYLIC ACID; TRIMETHOPRIM; METHYLMALONYL COENZYME A DECARBOXYLASE;

ADOLESCENT; AEROBIC METABOLISM; ARTICLE; CARDIOMYOPATHY; CHILD; CITRIC ACID CYCLE; CLINICAL ARTICLE; CONTROLLED STUDY; DETOXIFICATION; DISORDERS OF MITOCHONDRIAL FUNCTIONS; ENZYME ACTIVITY; FAILURE TO THRIVE; FEMALE; FIBROBLAST; HEART; HEART DILATATION; HEART FAILURE; HEMODIALYSIS; HUMAN; HUMAN TISSUE; IN VITRO STUDY; INFANT; KIDNEY; KIDNEY FAILURE; LIVER; MALE; METABOLIC ACIDOSIS; METABOLISM; METHYLMALONIC ACIDURIA; MITOCHONDRIAL OXIDATIVE PHOSPHORYLATION DEFICIENCY; MUSCLE HYPOTONIA; NEUROLOGIC DISEASE; OXIDATIVE STRESS; PARENTERAL NUTRITION; PRIORITY JOURNAL; PROPIONIC ACIDEMIA; PSYCHOMOTOR RETARDATION; RESPIRATORY CHAIN; SCHOOL CHILD; SKELETAL MUSCLE; VOMITING; WALKING DIFFICULTY; DISORDERS OF AMINO ACID AND PROTEIN METABOLISM; DISORDERS OF LIPID AND LIPOPROTEIN METABOLISM; ELECTRON TRANSPORT; ENZYMOLOGY; GENETICS; HEART MUSCLE; MUTATION; NEWBORN; OXIDATIVE PHOSPHORYLATION; PHYSIOLOGY;

ADOLESCENT; AMINO ACID METABOLISM, INBORN ERRORS; CARDIOMYOPATHIES; CITRIC ACID CYCLE; ELECTRON TRANSPORT; FEMALE; FIBROBLASTS; HUMANS; INFANT; INFANT, NEWBORN; KIDNEY; KIDNEY FAILURE; LIPID METABOLISM, INBORN ERRORS; LIVER; MALE; METHYLMALONYL-COA DECARBOXYLASE; MUSCLE, SKELETAL; MUTATION; MYOCARDIUM; NERVOUS SYSTEM DISEASES; OXIDATIVE PHOSPHORYLATION; OXIDATIVE STRESS; REACTIVE OXYGEN SPECIES;

EID: 70149095362     PISSN: 00313998     EISSN: 15300447     Source Type: Journal    
DOI: 10.1203/PDR.0b013e3181a7c270     Document Type: Article

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