Argininosuccinate synthetase

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Argininosuccinate Synthetase

Argininosuccinate synthetase (pronounced: ar-gi-ni-no-suc-ci-nate syn-thet-ase) is an enzyme that plays a crucial role in the urea cycle, which is the process by which mammals excrete ammonia.

Etymology

The term "Argininosuccinate synthetase" is derived from the name of the molecule it helps to produce, argininosuccinate, and the term "synthetase," which is used to denote enzymes that catalyze the bonding of two molecules.

Function

Argininosuccinate synthetase catalyzes the reaction of citrulline and aspartate to form argininosuccinate, with the help of ATP. This is a key step in the urea cycle, which helps to remove toxic ammonia from the body.

Related Terms

Disorders

Deficiency in argininosuccinate synthetase can lead to a rare genetic disorder known as argininosuccinate synthetase deficiency, or citrullinemia type I. This condition can cause an excess of ammonia in the blood, leading to various symptoms such as poor growth, developmental delay, and seizures.

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