| 1 | BBP-671 | - | - | - | - | [2]  245 245, 246 💬
 | 
| 2 | Carbaglu | [1]  Carglumic acid Carglumic acid | [1]  Carglumic acid 
                
                    Carglumic acid
                
             💬
 | [1]  CPS1 CPS1 💬
 | [6]  Alanine, aspartate and glutamate metabolism Alanine, aspartate and glutamate metabolism, Arginine biosynthesis, Biosynthesis of amino acids, Carbon metabolism, Metabolic pathways, Nitrogen metabolism 💬 | [2]  245 245, 246 💬
 | 
| 3 | Carglumic Acid | [1]  Carglumic acid Carglumic acid | [1]  Carglumic acid 
                
                    Carglumic acid
                
             💬
 | [1]  CPS1 CPS1 💬
 | [6]  Alanine, aspartate and glutamate metabolism Alanine, aspartate and glutamate metabolism, Arginine biosynthesis, Biosynthesis of amino acids, Carbon metabolism, Metabolic pathways, Nitrogen metabolism 💬 | [3]  245 245, 246, 251 💬
 | 
| 4 | Carglumic Acid (Carbaglu®) | [1]  Carglumic acid Carglumic acid | [1]  Carglumic acid 
                
                    Carglumic acid
                
             💬
 | [1]  CPS1 CPS1 💬
 | [6]  Alanine, aspartate and glutamate metabolism Alanine, aspartate and glutamate metabolism, Arginine biosynthesis, Biosynthesis of amino acids, Carbon metabolism, Metabolic pathways, Nitrogen metabolism 💬 | [2]  245 245, 246 💬
 | 
| 5 | CX-016492 | - | - | - | - | [1]  245 245 💬
 | 
| 6 | CX-017950 | - | - | - | - | [1]  245 245 💬
 | 
| 7 | Disodium citrate | - | - | - | - | [1]  245 245 💬
 | 
| 8 | Glutamine | [1]  L-Glutamine L-Glutamine | [1]  Glutamine 
                
                    Glutamine
                
             💬
 | - | - | [8]  87 87, 89, 96, 97, 113, 222, 245, 299 💬
 | 
| 9 | HST5040 | - | - | - | - | [2]  245 245, 246 💬
 | 
| 10 | MRNA-3927 | - | - | - | - | [1]  245 245 💬
 | 
| 11 | N-Carbamylglutamate | [1]  Carglumic acid Carglumic acid | [1]  Carglumic acid 
                
                    Carglumic acid
                
             💬
 | [1]  CPS1 CPS1 💬
 | [6]  Alanine, aspartate and glutamate metabolism Alanine, aspartate and glutamate metabolism, Arginine biosynthesis, Biosynthesis of amino acids, Carbon metabolism, Metabolic pathways, Nitrogen metabolism 💬 | [2]  245 245, 246 💬
 | 
| 12 | Ornithine | [1]  Ornithine Ornithine | [1]  Ornithine 
                
                    Ornithine
                
             💬
 | - | - | [2]  245 245, 251 💬
 | 
| 13 | Ornithine alpha ketoglutarate | [1]  Ornithine Ornithine | [1]  Ornithine 
                
                    Ornithine
                
             💬
 | - | - | [1]  245 245 💬
 | 
| 14 | Standard of Care Treatment | - | - | - | - | [3]  53 53, 245, 246 💬
 |