Argininosuccinate lyase

The argininosuccinate lyase (ASL), the enzyme that catalyzes the cleavage of arginine to argininosuccinate and fumarate. This reaction takes place in all living beings, rather than as the last step of arginine biosynthesis, but also counts in vertebrates as a partial step of the urea cycle. Mutations in the ASL gene can cause ASL deficiency, a urea cycle defect with excretion of arginine Oberstein acid in the urine.

ASL is due to its specific expression as a lab value for liver diseases.

Catalyzed reaction

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Argininosuccinate divided into fumarate and arginine.

Human Genome Organisation Digital Object Identifier
76369
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