Guanidinoacetate Methyltransferase Deficiency (GAMT Deficiency)
Metabolites
Enzyme
| EC Number | name | full name | note |
|---|---|---|---|
| 1.14.11.2 | procollagen-proline 4-dioxygenase | procollagen-L-proline,2-oxoglutarate:oxygen oxidoreductase (4-hydroxylating) | |
| 1.2.1.3 | aldehyde dehydrogenase (NAD+) | aldehyde:NAD+ oxidoreductase | |
| 1.4.1.3 | glutamate dehydrogenase [NAD(P)+] | L-glutamate:NAD(P)+ oxidoreductase (deaminating) | |
| 1.4.3.3 | D-amino-acid oxidase | D-amino-acid:oxygen oxidoreductase (deaminating) | |
| 1.5.1.2 | pyrroline-5-carboxylate reductase | L-proline:NAD(P)+ 5-oxidoreductase | |
| 1.5.5.2 | proline dehydrogenase | L-proline:quinone oxidoreductase | |
| 1.5.5.3 | hydroxyproline dehydrogenase | trans-4-hydroxy-L-proline:quinone oxidoreductase | |
| 2.1.1.2 | guanidinoacetate N-methyltransferase | S-adenosyl-L-methionine:N-guanidinoacetate methyltransferase | |
| 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 |
|---|