Pivotal role of inter-organ aspartate metabolism for treatment of mitochondrial aspartate-glutamate carrier 2 (citrin) deficiency, based on the mouse model

Previous studies using citrin/mitochondrial glycerol-3-phosphate (G3P) dehydrogenase (mGPD) double-knockout mice have demonstrated that increased dietary protein reduces the extent of carbohydrate-induced hyperammonemia observed in these mice. This study aimed to further elucidate the mechanisms of...

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Veröffentlicht in:	Scientific reports 2019-03, Vol.9 (1), p.4179-4179, Article 4179
Hauptverfasser:	Saheki, Takeyori, Moriyama, Mitsuaki, Kuroda, Eishi, Funahashi, Aki, Yasuda, Izumi, Setogawa, Yoshiko, Gao, Qinghua, Ushikai, Miharu, Furuie, Sumie, Yamamura, Ken-ichi, Takano, Katsura, Nakamura, Yoichi, Eto, Kazuhiro, Kadowaki, Takashi, Sinasac, David S., Furukawa, Tatsuhiko, Horiuchi, Masahisa, Tai, Yen How
Format:	Artikel
Sprache:	eng
Schlagworte:	631/45 64 64/110 64/60 692/699/317 Alanine Amino acids Amino Acids - blood Amino Acids - pharmacology Ammonia Ammonia - blood Ammonium Chloride - metabolism Animals Arginine Aspartic Acid - metabolism Citrulline Citrulline - pharmacology Citrullinemia - metabolism Disease Models, Animal Ethanol Glycerol Glycerol-3-phosphate Glycerolphosphate Dehydrogenase - metabolism Glycine Humanities and Social Sciences Hyperammonemia Hyperammonemia - blood Intestine, Small - metabolism Lactates - metabolism Liver Liver - metabolism Metabolism Mice, Inbred C57BL Mice, Knockout Mitochondria Mitochondrial Membrane Transport Proteins - deficiency multidisciplinary NAD NADH Optics Oral administration Organ Specificity Ornithine Ornithine - pharmacology Perfusion Physics Portal vein Portal Vein - metabolism Pyruvic Acid - metabolism Rodents Science Science (multidisciplinary) Small intestine Sucrose Triglycerides Urea - metabolism
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