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Ammonia metabolism pathways. A, Urea cycle. B, Pyrimidine biosynthesis. C, Krebs cycle anaplerosis pathways. Green boxes mark key enzymes, dotted lines encircle steps inside mitochondria, and red stars mark defects in the patient. Additional abbreviations: DHO, dihydroorotate; MUT, methylmalonyl‐CoA mutase; NAG, N‐acetylglutamate; NAGS, N‐acetylglutamate synthase; OMP, orotidine monophosphate; PCC, propionyl‐CoA carboxylase; RR, ribonucleotide reductase; TS, thymidylate synthase
