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Canalicular multispecific organic anion transporter 2 Solute carrier family 22 member 5 Mitochondrial carnitine/acylcarnitine carrier protein CACL Neutral amino acid transporter A Calcium-binding mitochondrial carrier protein Aralar1 Multidrug resistance- associated protein 4 Mitochondrial ornithine transporter 1 Delta-1- pyrroline-5- carboxylate synthase Glutaminase liver isoform, mitochondrial Ornithine aminotransferase, mitochondrial Ornithine carbamoyltransferase, mitochondrial Argininosuccinate synthase Argininosuccinate lyase Arginase, mitochondrial D-amino-acid oxidase Unknown Argininic acid L-Glutamine L-Glutamine L-Glutamine Citrulline Citrulline L-Glutamine Argininic acid Ornithine Ornithine L-Glutamic acid NADPH H+ 1-Pyrroline-5-carboxylic acid NADP H2O NH3 Glutaric acid Oxoglutaric acid Carbamoyl phosphate Pi ATP L-Aspartic acid AMP PPi Argininosuccinic acid Fumaric acid L-Arginine H2O Urea NADP H2O 2-Oxoarginine NADPH NH3 NADPH NADP Calcium Pyridoxal 5'-phosphate FAD Neurotoxicity Glutamine is ingested through food such as beef, pork, poultry, dairy, spinach, parsley, and cabbage. Intestine Intestine Epithelial Cell Blood vessel Hepatocyte Mitochondria Mitochondrial Matrix Cytosol Low concentrations of Arginase causes an accumulation of Arginine, and prevents the synthesis of Urea and Ornithine. Argininic acid is synthesized by an unknown hydrogenase Argininic acid is a uremic toxin which causes neurological syndrome consisting of a variable degree of mental retardation, epilepsy and progressive spasticity.