SAPROPTERIN (is an active metabolite) 
| SAPROPTERIN SEPIAPTERIN | NATURAL MOLECULE (link) ATC A16AX07
METABOLISM AGONIST TREATMENT OF HYPERPHENYLALANINAEMIA (HPA) REDUCTION OF PHENYLALANINE CONCENTRATION IN PATIENTS WITH PHENYLKETONURIA (PKU) COFACTOR FOR THE ENZYME PHENYLALANINE HYDROXYLASE (PAH). TREATMENT WITH BH4 CAN ACTIVATE RESIDUAL PAH AND IMPROVE THE NORMAL OXIDATIVE METABOLISM OF PHE, AND DECREASE PHE LEVELS TREATMENT OF TETRAHYDROBIOPTERIN (BH4) DEFICIENCY | ORAL | Cmax 1.79 MICROMOLAR Tmax 3.5 HOUR VD 11433 LITER PPB 32.7 PERCENT Cl 1498 LITER / HOUR HT 6.7 HOUR SOLUBILITY SOLUBLE IN WATER | PHENYLALANINE HYDROXYLASE PDB 8XZN (CRYSTAL STRUCTURE OF FOLE RIBOSWITCH WITH BH4) LIGAND CODE = H4B (link to the list of PDB complexes) Download experimental 3D coordinates of H4B with added hydrogens | Phenylalanine-4-hydroxylase UNIPROT P00439 PAH more at DrugCentral | EMA ANSM (in French) Inxight Drugs Dailymed Drugs.com SIDER side effects Chemical Probes Portal ChEMBL BindingDB DrugBank |