Carbamoyl Phosphate Synthetase Deficiency
Metabolites
Enzyme
| EC Number | name | full name | note |
|---|---|---|---|
| 1.4.1.3 | glutamate dehydrogenase [NAD(P)+] | L-glutamate:NAD(P)+ oxidoreductase (deaminating) | |
| 2.1.3.3 | ornithine carbamoyltransferase | carbamoyl-phosphate:L-ornithine carbamoyltransferase | |
| 2.6.1.1 | aspartate transaminase | L-aspartate:2-oxoglutarate aminotransferase | |
| 2.6.1.2 | alanine transaminase | L-alanine:2-oxoglutarate aminotransferase | |
| 2.6.1.7 | kynurenine-oxoglutarate transaminase | L-kynurenine:2-oxoglutarate aminotransferase | |
| 3.5.1.2 | glutaminase | L-glutamine amidohydrolase | |
| 3.5.3.1 | arginase | L-arginine amidinohydrolase | |
| 4.3.2.1 | argininosuccinate lyase | 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming) | |
| 6.3.4.16 | carbamoyl-phosphate synthase (ammonia) | carbon-dioxide:ammonia ligase (ADP-forming, carbamate-phosphorylating) | |
| 6.3.4.5 | argininosuccinate synthase | L-citrulline:L-aspartate ligase (AMP-forming) |
Proteins
| Protein ID | name | full name |
|---|