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Biochemistry, cell biology, and genetics

Pyrimidine synthesis and nucleotide disorders

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SAMPLE QUESTION

A 22-year-old man develops vomiting and confusion after beginning a high-protein diet. His family recalls similar, milder episodes during illnesses. Plasma ammonia is 318 µmol/L, plasma citrulline is markedly reduced, and urinary orotic acid is markedly increased. Hemoglobin and mean corpuscular volume are normal. Emergency treatment for hyperammonemia is begun.

The excess carbamoyl phosphate supplying orotic acid synthesis in this patient is most likely formed in which location and from which nitrogen donor?