argininosuccinate <=> fumarate + arginine

Cytosolic argininosuccinate lyase (ASL) catalyzes the reversible reaction of argininosuccinate to form fumarate and arginine. The enzyme is a homotetramer (Turner et al. 1997). The function of the human enzyme in vivo is inferred from the defective argininosuccinate lyase enzyme activity observed in patients with mutant forms of the ASL gene (e.g., Walker et al. 1990).

细胞质中的精氨酸琥珀酸裂解酶（ASL）催化精氨酸琥珀酸的可逆反应，生成富马酸和精氨酸。该酶为同源四聚体（Turner 等人，1997年）。人类酶在体内的功能可从患有ASL基因突变型患者中观察到的缺陷型精氨酸琥珀酸裂解酶活性推断得出（例如，Walker 等人，1990年）。