Homocystinuria, Cystathionine beta-Synthase Deficiency
Metabolites
Enzyme
| EC Number | name | full name | note |
|---|---|---|---|
| 4.2.1.22 | cystathionine beta-synthase | L-serine hydro-lyase (adding homocysteine; L-cystathionine-forming) | |
| 4.4.1.1 | cystathionine gamma-lyase | L-cystathionine cysteine-lyase (deaminating; 2-oxobutanoate-forming) | |
| 4.4.1.2 | homocysteine desulfhydrase | L-homocysteine hydrogen-sulfide-lyase (deaminating; 2-oxobutanoate-forming) |
Proteins
| Protein ID | name | full name |
|---|