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argininosuccinase
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argininosuccinase

enzyme
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Quick Summary

Argininosuccinase is an enzyme that plays a crucial role in the urea cycle, a metabolic pathway in mammals responsible for detoxifying ammonia. This enzyme catalyzes the splitting of argininosuccinate into fumarate and arginine. Arginine is an amino acid that is essential for the synthesis of urea, the primary form in which mammals excrete nitrogen. Defects in the urea cycle, including those involving argininosuccinase, can lead to a toxic accumulation of ammonia in the blood, a condition known as urea cycle disorder.123

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5 Britannica Sources
  1. metabolism - Disposal of nitrogen
  2. arginine
  3. metabolic disease - Urea cycle defects
  4. arginase
  5. metabolic disease - Amino acid transport disorders

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