In argininosuccinase deficiency, what should be supplemented to continue the urea cycle ?
Argininosuccinase (argininosuccinate lyase) catalyses the cleavage of arginiosuccinate into arginine and fumarate. o Thus, in argininosuccinase deficiency arginine cannot be produced. Supplementation with arginine base helps replenish this amino acid.
Aspaate
Arginine
Citrullin
Argininosuccinate