- 英文名称
- Sapropterin dihydrochloride
- 分子式
- C9H17Cl2N5O3
- 分子量
- 314.17
- 中文别名
- (R)-2-氨基-6-((1R,2S)-1,2-二羟丙基)-5,6,7,8-四氢蝶呤-4(1H)-酮 二盐酸盐; 四氢生物蝶呤; (6R)-5,6,7,8-四氢-L-生物喋呤二盐酸盐; 二盐酸(6R)-5,6,7,8-四氢生物蝶呤; 沙丙蝶呤; 盐酸沙丙蝶呤; 四氢-L-生物喋呤; 沙丙蝶呤二盐酸盐; 四氢生物蝶呤二盐酸盐; 四氢生物喋呤二盐酸盐; (R)-2-Amino-6-((1R,2S)-1,2-dihydroxypropyl)-5,6,7,8-tetrahydropteridin-4(1H)-one dihydrochloride; (6R)-2-amino-6-[(1R,2S)-1,2-dihydroxypropyl]-5,6,7,8-tetrahydropteridin-4(1H)-one dihydrochloride; (6R,1'R,2'S)-6-(1',2'-dihydroxypropyl)-5,6,7,8-tetrahydrobiopterin; Dapropterin; (6R)-2-Amino-6-[(1R,2S)-1,2-dihydroxypropyl]-5,6,7,8-tetrahydro-4(1H)-pteridinone; (6R)-5,6,7,8-tetrahydro-L-biopterin; (6R)-Tetrahydrobiopterin; 6R-tetahydrobiopterin; Dapropterin dihydrochloride; Biopten; sapropterinum; R-THBP; (R)-2-Amino-6-((1R,2S)-1,2-dihydroxypropyl)-5,6,7,8-tetrahydropteridin-4(3H)-one dihydrochloride; [6R-[6R*(1R,2S*)]]-2-amino-6-(1,2-dihydroxypropyl)-5,6,7,8-tetrahydro-4(1H)-pteridinone; (6R)-Tetrahydrobiopterin hydrochloride; Sapropterin; kuvan; 6b-5,6,7,8-Tetrahydro-L-biopterin; 6R-Tetrahydro-L-biopterin; R-THBP6R-BH4
- 用途
- 作为苯丙氨酸羟化酶、酪氨酸羟化酶、色氨酸羟化酶、一氧化氮合酶和烷基甘油单加氧酶的天然辅助因子；是治疗四氢生物蝶呤(BH4)缺乏症所导致的高苯丙氨酸血症(HPA)的唯一药物，适用于成人以及4岁以上儿童。
