Ornithine carbamoyltransferase, mitochondrial
Aspartate aminotransferase, mitochondrial
L-Glutamine (→) Transport: Homo sapiens, Extracellular Space to Homo sapiens, Cell, Cytoplasm
L-Alanine (→) Transport: Homo sapiens, Extracellular Space to Homo sapiens, Cell, Cytoplasm
Carbamoyl-phosphate synthase [ammonia], mitochondrial
L-Arginine + Water → Ornithine + Urea
Calcium-binding mitochondrial carrier protein Aralar1
L-Glutamic acid + NAD + Water → Ammonia + NADH + Oxoglutaric acid
L-Glutamine + Water → Ammonia + L-Glutamic acid
2 Argininosuccinic acid → Fumaric acid + L-Arginine
Adenosine triphosphate + Citrulline + L-Aspartic acid → Adenosine monophosphate + Argininosuccinic acid + Pyrophosphate
Glutamate dehydrogenase 1, mitochondrial
L-Alanine + Oxoglutaric acid ↔ L-Glutamic acid + Pyruvic acid
Glutaminase liver isoform, mitochondrial
Carbamoyl phosphate + Ornithine → Citrulline + Phosphate
L-Aspartic acid + Oxoglutaric acid ↔ L-Glutamic acid + Oxalacetic acid
Ornithine Transcarbamylase Deficiency (OTC Deficiency)
Ornithine (→), Citrulline (←) Transport: Homo sapiens, Cell, Cytoplasm to Homo sapiens, Cell, Mitochondrial Matrix
L-Aspartic acid (→), L-Glutamic acid (←) Transport: Homo sapiens, Cell, Mitochondrial Matrix to Homo sapiens, Cell, Mitochondrial Intermembrane Space
2 Adenosine triphosphate + Ammonia + Carbon dioxide + Water → 2 Adenosine diphosphate + Carbamoyl phosphate + Phosphoric acid