Question

can you have a problem in the urea cycle without having hyperammonemia?

can you have a problem in the urea cycle without having hyperammonemia?

Homework Answers

Answer #1

Metabolic defects in the urea cycle invariably lead to hyperammonemia. But other metabolites in the urea cycle also accumulate if there is any defect involving the enzyme that catalyses the conversion of that metabolite.

For example defect in Arginosuccinate synthase causes citrullinemia, defect in arginosuccinase causes arginosuccinic aciduria and defect in arginase causes hyperargininemia.

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