Argininic acid is an uremic toxin produced in people with hyperargininemia. Hyperargininemia is an inborn problem in the urea cycle caused by a deficiency in liver arginase. Glutamine is consumed through food and taken into the blood through the intestines. It is transported to the liver where it is transported in via an amino acid transport. Glutamine is then transported into the mitochondria by the transporter glutamate antiporter SLC25A12, mitochondrial. In the mitochondria it is catalyzed by Glutaminase liver isoform, mitochondrial into glutamic acid. Glutamic acid is carboxylated into 1-Pyrroline-5-carboxylic acid by the enzyme Delta-1-pyrroline-5-carboxylate synthase. 1-Pyrroline-5-carboxylic acid with glutaric acid synthesize Oxoglutaric acid and ornithine with the enzyme Ornithine aminotransferase, mitochondrial. Ornithine and Carbamoyl phosphate are catalyzed by the enzyme ornithine carbamoyltransferase, mitochondrial to synthesize citrulline. Citrulline is transported out of the mitochondria into the cytosol by a mitochondrial transporter. Arginnosuccinate is synthesized from citrulline and aspartic acid by the enzyme argininosuccinate synthase. That is catalyzed by argininosuccinate lyase to produce arginine and fumaric acid. In a healthy individual arginine would then continue in the urea cycle to synthesize urea and ornithine, however, due to the deficiency in arginase, arginine is accumulates in the liver, causing hyperargininemia. This accumulation causes arginine to instead be converted into α-keto-δ-guanidinovaleric acid by transamination. α-Keto-δ-guanidinovaleric acid is then hydrogenated to form argininic acid. Due to the accumulation of arginine, argininic acid is present in high concentrations. Argininic acid is a uremic toxin that is toxic to the nervous system, and it causes various neurlogical disorders such as variable degree of mental retardation, epilepsy and progressive spasticity.

Metabolsim and Physiological Effects of Argininic acid References