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四氢生物蝶呤

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四氢生物蝶呤
(6R)-Tetrahydrobiopterin structure.svg
临床资料
核准状况
给药途径Oral
ATC码
法律规范状态
法律规范
识别信息
CAS号17528-72-2  ✓
PubChem CID
IUPHAR/BPS
DrugBank
ChemSpider
ChEBI
ChEMBL
CompTox Dashboard (EPA)
ECHA InfoCard100.164.121 编辑维基数据链接
化学信息
化学式C9H15N5O3
摩尔质量241.25

四氢生物蝶呤tetrahydrobiopterin),又称沙丙蝶呤Sapropterin),简称BH4, 是三种芳香族氨基酸羟化酶的一种重要辅酶苯丙氨酸羟化酶苯丙氨酸转化为酪氨酸酪氨酸羟化酶酪氨酸转化为左旋多巴色氨酸羟化酶色氨酸转化为5-羟色胺酸。它也是一氧化氮合酶(NOS)合成NO的重要辅酶。

临床意义

四氢生物蝶呤合成和/或再生障碍可导致IV型苯丙酮尿症(PKU)以及神经递质多巴胺5-羟色胺)。PKU患者血液里长期高浓度的苯丙氨酸可导致严重的神经损害,包括严重智力缺陷、小颅、言语延迟、癫痫和行为异常。

生物合成

四氢生物蝶呤由GTP经三次酶促反应(GTP环化水解酶I、6-丙酮酰四氢生物蝶呤合成酶和 乌蝶呤还原酶)合成[1]

参考文献

  1. ^ Thony B, Auerbach G, Blau N. Tetrahydrobiopterin biosynthesis, regeneration and functions. Biochem J. 2000,. 347 Pt 1: 1–16 [2009-01-20]. PMID 10727395. doi:10.1042/0264-6021:3470001. (原始内容存档于2006-05-08). 
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四氢生物蝶呤
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