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- ASL : argininosuccinate lyase
- CPS1 : carbamoyl-phosphate synthase 1
- ASS1 : argininosuccinate synthase 1
- PYCR1 : pyrroline-5-carboxylate reductase 1
- AASS : aminoadipate-semialdehyde synthase
- SLC25A1 : solute carrier family 25 member 1
- NAGS : N-acetylglutamate synthase
- HADHB : hydroxyacyl-CoA dehydrogenase trifunctional multienzyme complex subunit beta
- Citrullinemia Type I