| Article ID | Journal | Published Year | Pages | File Type |
|---|---|---|---|---|
| 10833810 | Molecular Genetics and Metabolism | 2011 | 9 Pages |
Abstract
⺠The citrin/mGPD double-knockout mouse is a model of human citrin deficiency. ⺠Sucrose administration caused an increase in hepatic glycerol 3-phosphate. ⺠Sucrose administration caused increases in hepatic citrulline and lysine. ⺠Sucrose administration also caused a decrease in TCA cycle intermediates. ⺠Sodium pyruvate ameliorated hyperammonemia and hepatic metabolite perturbations.
Keywords
ASsnicotinamide adenine dinucleotide, reducedMGpdCE/MSadult-onset type II citrullinemiaargininosuccinateAASSαKGPYRNeonatal intrahepatic cholestasis caused by citrin deficiencyNICCDCTLN2AGCGLNORNALAASPG3PDHAPTCAASALACGC/MSLC/MSNAD+α-ketoglutarateArginineargininosuccinate synthetaseaspartatealanineArgtricarboxylic acidornithineanalysis of varianceANOVAaspartate–glutamate carrierdihydroxyacetone phosphateSodiumCitrinCitrullineLactateLysineLYSNADHknockoutwild-typePyruvateSodium pyruvateLiquid chromatography/mass spectrometrygas chromatography/mass spectrometryGluglutamateglutamineglycerol-3-phosphatemitochondrial glycerol-3-phosphate dehydrogenase
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Authors
Takeyori Saheki, Kanako Inoue, Hiromi Ono, Anmi Tushima, Natsumi Katsura, Mana Yokogawa, Yukari Yoshidumi, Tomiko Kuhara, Morimasa Ohse, Kazuhiro Eto, Takashi Kadowaki, David S. Sinasac, Keiko Kobayashi,