AP - Arginosuccinic Aciduria

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Last updated 12:47 AM on 9/26/26
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4 Terms

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argininosuccinic acid lyase

Arginoccucinic Aciduria lack the enzyme

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arginine

argininosuccinic acid lyase, which prevents the conversion of argininosuccinic acid into

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citrulline & ammonia in blood

Elevated levels of argininosuccinic acid also cause buildup of

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  1. High-caloric, protein-restrictive diet

  2. Arginine supplementation

  3. Administration of sodium benzoate and sodium phenylacetate


What is the treatment of Arginoccucinic aciduria