A Liver-Specific Defect of Acyl-CoA Degradation Produces Hyperammonemia, Hypoglycemia and a Distinct Hepatic Acyl-CoA Pattern

PLoS ONE

Volumn 8, Issue 7, 2013, Pages

  Gauthier, Nicolas ^a,b   Wu, Jiang Wei ^a   Wang, Shu Pei ^a   Allard, Pierre ^a   Mamer, Orval A ^c   Sweetman, Lawrence ^d   Moser, Ann B ^e   Kratz, Lisa ^e   Alvarez, Fernando ^a   Robitaille, Yves ^a   Lépine, François ^f   Mitchell, Grant A ^a,b  

^a UNIVERSITY OF MONTREAL   (Canada)

^b UNIVERSITÉ DE MONTRÉAL   (Canada)

^c MCGILL UNIVERSITY   (Canada)

^d BAYLOR UNIVERSITY MEDICAL CENTER   (United States)

^e JOHNS HOPKINS UNIVERSITY SCHOOL OF MEDICINE   (United States)

^f INRS INSTITUT ARMAND FRAPPIER   (Canada)

Author keywords

[No Author keywords available]

Indexed keywords

2 OXOISOCAPROIC ACID; ACYL COENZYME A; ACYLCARNITINE; GLUTAMIC ACID; HYDROXYMETHYLGLUTARYL COENZYME A LYASE; KETONE BODY; LEUCINE; UREA;

ANIMAL CELL; ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; CARBOXYLATION; CATALYSIS; CONTROLLED STUDY; ENZYME DEGRADATION; GLUCONEOGENESIS; HYPERAMMONEMIA; HYPERGLYCEMIA; HYPOGLYCEMIA; INBORN ERROR OF METABOLISM; KETOGENESIS; LIVER; LIVER CELL; MITOCHONDRION; MOUSE; NONHUMAN; PATHOPHYSIOLOGY; UREA CYCLE;

ACETYL COENZYME A; ACYL COENZYME A; ANIMALS; CARBON DIOXIDE; GENE KNOCKOUT TECHNIQUES; GENE ORDER; GENE TARGETING; GENES, LETHAL; GLUCONEOGENESIS; HEPATOCYTES; HUMANS; HYPERAMMONEMIA; HYPOGLYCEMIA; LETHARGY; LEUCINE; LIVER; METABOLIC NETWORKS AND PATHWAYS; METABOLOME; MICE; MICE, KNOCKOUT; MITOCHONDRIA; MODELS, BIOLOGICAL; PEROXISOMES; PHENOTYPE; PYRUVIC ACID;

MUS;

EID: 84879821655     PISSN: None     EISSN: 19326203     Source Type: Journal    
DOI: 10.1371/journal.pone.0060581     Document Type: Article

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