Chapter 26, Problem 26.67EP

Interpretation Introduction

Interpretation: To determine the step(s) of the urea cycle that occurs in the mitochondrial matrix.

Concept introduction: Urea cycle constitutes 4 steps. In step 1 carbamoyl phosphate transfers its carbamoyl group to ornithine to produce citrulline. Citrulline then condenses with aspartate in the second step to form argininosuccinate which then cleaves in the third step to form arginine and fumarate. In the final step, arginine reacts with water to produce urea.