Metabolomic analysis reveals hepatic metabolite perturbations in citrin/mitochondrial glycerol-3-phosphate dehydrogenase double-knockout mice, a model of human citrin deficiency

Molecular Genetics and Metabolism

Volumn 104, Issue 4, 2011, Pages 492-500

  Saheki, Takeyori ^a,h   Inoue, Kanako ^a   Ono, Hiromi ^a   Tushima, Anmi ^a   Katsura, Natsumi ^a   Yokogawa, Mana ^a   Yoshidumi, Yukari ^a   Kuhara, Tomiko ^b   Ohse, Morimasa ^b   Eto, Kazuhiro ^c   Kadowaki, Takashi ^d   Sinasac, David S ^e,f   Kobayashi, Keiko ^g  

^a TOKUSHIMA BUNRI UNIVERSITY   (Japan)

^b KANAZAWA MEDICAL UNIVERSITY   (Japan)

^c TEIKYO UNIVERSITY   (Japan)

^d UNIVERSITY OF TOKYO   (Japan)

^e ALBERTA CHILDREN'S HOSPITAL   (Canada)

^f UNIVERSITY OF CALGARY   (Canada)

^g KAGOSHIMA UNIVERSITY   (Japan)

^h KUMAMOTO UNIVERSITY   (Japan)

Author keywords

Adult onset type II citrullinemia (CTLN2); Citrin; Citrulline; Glycerol 3 phosphate; Sodium pyruvate

Indexed keywords

AMINO ACID; CITRULLINE; ERIODICTYOL 7 O GLUCOSIDE; GLYCEROL 3 PHOSPHATE DEHYDROGENASE; LYSINE; MITOCHONDRIAL ENZYME; PYRUVATE SODIUM; SUCROSE; TRICARBOXYLIC ACID; UNCLASSIFIED DRUG;

AMINO ACID METABOLISM; ANIMAL EXPERIMENT; ANIMAL MODEL; ARTICLE; CAPILLARY ELECTROPHORESIS; CATABOLISM; CITRIN DEFICIENCY; CONTROLLED STUDY; ENZYME ANALYSIS; ENZYME DEFICIENCY; HYPERAMMONEMIA; KNOCKOUT MOUSE; LIVER METABOLISM; MASS FRAGMENTOGRAPHY; MASS SPECTROMETRY; METABOLIC DISORDER; METABOLOMICS; MOUSE; NONHUMAN; PHENOTYPE; PRIORITY JOURNAL; UREA CYCLE; WILD TYPE;

AMMONIA; ANIMALS; CALCIUM-BINDING PROTEINS; CITRIC ACID CYCLE; DISEASE MODELS, ANIMAL; ELECTROPHORESIS, CAPILLARY; GAS CHROMATOGRAPHY-MASS SPECTROMETRY; GLYCEROLPHOSPHATE DEHYDROGENASE; GLYCOLYSIS; HUMANS; LIVER; METABOLOME; METABOLOMICS; MICE; MICE, INBRED C57BL; MICE, KNOCKOUT; MITOCHONDRIA; ORGANIC ANION TRANSPORTERS; PYRUVIC ACID; SUCROSE; UREA;

MUS;

EID: 82255179500     PISSN: 10967192     EISSN: 10967206     Source Type: Journal    
DOI: 10.1016/j.ymgme.2011.08.015     Document Type: Article

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