Argininosuccinic Acid Unit Converter

Arginosuccinate (argininosuccinic acid) is an amino acid intermediate in the urea cycle, the process your liver uses to convert toxic ammonia into urea for excretion. It is not one of the standard protein-building amino acids, but rather a metabolic intermediate.

In the urea cycle, arginosuccinate is formed from citrulline and aspartate by the enzyme argininosuccinate synthetase. It is then broken down by argininosuccinate lyase into arginine and fumarate. Measuring arginosuccinate in blood or urine helps evaluate how well this pathway is functioning.

Under normal conditions, arginosuccinate is present in very low or undetectable amounts in plasma. Elevated levels are a hallmark finding for argininosuccinate lyase deficiency, the second most common urea cycle disorder.

Arginosuccinate is produced in the liver as part of the urea cycle, formed when the enzyme argininosuccinate synthetase combines citrulline with aspartate.