argininosuccinic acidemia

argininosuccinic acidemia

[ärjin′inō′suksin′ik]
an inherited amino acid metabolism disorder in which the lack of an enzyme, argininosuccinase, results in an excess of argininosuccinic acid in the blood. The condition is characterized by seizures and mental retardation. Treatment mainly involves a low-protein diet containing essential amino acids or amino acid analogs.
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