Drug Name |
Sapropterin dihydrochloride |
Drug ID |
BADD_D01988 |
Description |
Sapropterin (tetrahydrobiopterin or BH4) is a cofactor in the synthesis of nitric oxide. It is also essential in the conversion of phenylalanine to tyrosine by the enzyme phenylalanine-4-hydroxylase; the conversion of tyrosine to L-dopa by the enzyme tyrosine hydroxylase; and conversion of tryptophan to 5-hydroxytryptophan via tryptophan hydroxylase. |
Indications and Usage |
For the treatment of tetrahydrobiopterin (BH4) deficiency. |
Marketing Status |
approved; investigational |
ATC Code |
A16AX07 |
DrugBank ID |
DB00360
|
KEGG ID |
D01798
|
MeSH ID |
C003402
|
PubChem ID |
135409471
|
TTD Drug ID |
D0Q9YT
|
NDC Product Code |
51846-1057; 43598-749; 49884-873; 49884-948; 68135-301; 69037-0048; 31722-045; 68135-482; 43598-477; 31722-047; 43598-097; 46438-0660; 31722-048; 66652-2103; 49884-720; 54864-872; 72643-026; 73435-017; 43598-096; 68135-300; 43598-162 |
UNII |
RG277LF5B3
|
Synonyms |
sapropterin | 6R-BH4 | tetrahydrobiopterin | 5,6,7,8-erythro-tetrahydrobiopterin | tetrahydro-6-biopterin | phenylalanine hydroxylase cofactor | 5,6,7,8-tetrahydrodictyopterin | D-threo-tetrahydrobiopterin | 5,6,7,8-tetrahydro-L-erythrobiopterin | sapropterin dihydrochloride | 2,4,5-trihydroxybutyrophenone | THBP | 1-butanone, 1-(2,4,5-trihydroxyphenyl) | trihydroxybutyrophenone | 2',4',5'-trihydroxybutyrophenone | 6R-L-erythro-5,6,7,8-tetrahydrobiopterin | 5,6,7,8-tetrahydrobiopterin, (S-(R*,S*))-isomer | 5,6,7,8-tetrahydrobiopterin | kuvan |