Urea cycle disorders

Double-stroked boxes represent the source of nitrogen for urea formation. Precursor amino acids contributing to the nitrogen pool include glutamine, glycine, and alanine.

CPS: carbamyl phosphate synthetase; OTC: ornithine transcarbamylase; ASS: argininosuccinate synthetase; ASL: argininosuccinic acid lyase.

Graphic 73454 Version 2.0

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