In argininosuccinase deficiency, what should be supplemented to continue the urea cycle ?: (A) Aspaate, (B) Arginine, (C) Citrullin, (D) Argininosuccinate
Answer is B. 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.