Argininosuccinic acid is a non-proteinogenic amino acid that is an important intermediate in the urea cycle. It is also known as argininosuccinate.

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Some cells synthesize argininosuccinic acid from citrulline and aspartic acid and use it as a precursor for arginine in the urea cycle (or citrulline-NO cycle), releasing fumarate as a by-product to enter the TCA cycle. The enzyme that catalyzes the reaction is argininosuccinate synthetase.

Argininosuccinic acid is a precursor to fumarate in the citric acid cycle via argininosuccinate lyase.

See also

  • Succinic acid

References


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Optimizing therapy for argininosuccinic aciduria. Semantic Scholar

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