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Sapropterin dihydrochloride(6R-BH4 dihydrochloride)
本产品不向个人销售,仅用作科学研究,不用于任何人体实验及非科研性质的动物实验。
Sapropterin dihydrochloride(6R-BH4 dihydrochloride)图片
CAS NO:69056-38-8
包装与价格:
包装价格(元)
10mM (in 1mL Water)电议
50mg电议
100mg电议

产品介绍
Sapropterin ((6R)-BH4) dihydrochloride 是一种具有口服活性的苯丙氨酸羟化酶 (PAH) 辅因子,可有效降低血液苯丙氨酸浓度。
Cas No.69056-38-8
别名盐酸沙丙蝶呤; (6R)-BH4 dihydrochloride; (6R)-Tetrahydro-L-biopterin dihydrochloride
Canonical SMILESO=C1N=C(N)NC2=C1N[C@@H]([C@@H](O)[C@@H](O)C)CN2.[H]Cl.[H]Cl
分子式C9H17Cl2N5O3
分子量314.17
溶解度DMSO : 63mg/mL, Water : 34 mg/mL
储存条件Store at -20°C
General tipsFor obtaining a higher solubility , please warm the tube at 37 ℃ and shake it in the ultrasonic bath for a while.
Shipping ConditionEvaluation sample solution : ship with blue ice
All other available size: ship with RT , or blue ice upon request
产品描述

Sapropterin dihydrochloride is a synthetic form of BH4 that is approved for the treatment of BH4 responsive PKU. (1) Sapropterin dihydrochloride can stimulate TH and TPH activities leading to improved dopamine and serotonin synthesis despite persistently elevated brain phenylalanine.(2) Sapropterin dihydrochloride is used to lower blood phenylalanine levels in tetrahydrobiopterin-responsive phenylketonuria in conjunction with a phenylalanine-restricted diet.

[1]. Winn SR et al. High dose sapropterin dihydrochloride therapy improves monoamine neurotransmitter turnover in murine phenylketonuria (PKU). Mol Genet Metab. 2016 Jan;117(1):5-11. [2]. Somaraju UR et al. Sapropterin dihydrochloride for phenylketonuria. Cochrane Database Syst Rev. 2015 Mar 27 [3]. Jurecki ER et al. Sapropterin Dihydrochloride Mixed With Common Foods and Beverages. Top Clin Nutr. 2014 Oct;29(4):325-331. [4]. Grange DK et al. Sapropterin dihydrochloride use in pregnant women with phenylketonuria: an interim report of the PKU MOMS sub-registry. Mol Genet Metab. 2014 May;112(1):9-16