Ureidopropionase Deficiency
Metabolites
3-[[(2S)-2,4-Dihydroxy-3,3-dimethylbutanoyl]amino]propanoic acid
Formula: C9H17NO5 (219.11066720000002)
CAS ID: 37138-77-5
Enzyme
| EC Number | name | full name | note |
|---|---|---|---|
| 1.2.1.27 | methylmalonate-semialdehyde dehydrogenase (CoA-acylating) | 2-methyl-3-oxopropanoate:NAD+ 3-oxidoreductase (CoA-propanoylating) | |
| 1.2.1.3 | aldehyde dehydrogenase (NAD+) | aldehyde:NAD+ oxidoreductase | |
| 1.3.1.2 | dihydropyrimidine dehydrogenase (NADP+) | 5,6-dihydrouracil:NADP+ 5-oxidoreductase | |
| 1.4.3.21 | primary-amine oxidase | primary-amine:oxygen oxidoreductase (deaminating) | |
| 2.6.1.19 | 4-aminobutyrate-2-oxoglutarate transaminase | 4-aminobutanoate:2-oxoglutarate aminotransferase | |
| 2.6.1.22 | (S)-3-amino-2-methylpropionate transaminase | (S)-3-amino-2-methylpropanoate:2-oxoglutarate aminotransferase | |
| 3.4.13.20 | beta-Ala-His dipeptidase | ||
| 3.5.1.6 | beta-ureidopropionase | N-carbamoyl-beta-alanine amidohydrolase | |
| 3.5.2.2 | dihydropyrimidinase | 5,6-dihydropyrimidine amidohydrolase | |
| 4.1.1.15 | glutamate decarboxylase | L-glutamate 1-carboxy-lyase (4-aminobutanoate-forming) |
Proteins
| Protein ID | name | full name |
|---|