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/ Sapropterin 2HCl/盐酸沙丙蝶呤

Sapropterin 2HCl/盐酸沙丙蝶呤 {[allProObj[0].p_purity_real_show]}

货号：A701042 同义名： (6R)-BH4 dihydrochloride; (6R)-Tetrahydro-L-biopterin dihydrochloride

Sapropterin 2HCl是一种苯丙氨酸羟化酶（PAH）激活剂，降低血液中的苯丙氨酸浓度，可用于研究苯丙酮尿症。

Sapropterin 2HCl/盐酸沙丙蝶呤 化学结构 CAS号：69056-38-8
Sapropterin 2HCl/盐酸沙丙蝶呤 化学结构
CAS号：69056-38-8
Sapropterin 2HCl/盐酸沙丙蝶呤 3D分子结构
CAS号：69056-38-8
Sapropterin 2HCl/盐酸沙丙蝶呤 化学结构 CAS号：69056-38-8
Sapropterin 2HCl/盐酸沙丙蝶呤 3D分子结构 CAS号：69056-38-8
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Sapropterin 2HCl/盐酸沙丙蝶呤 纯度/质量文件 产品仅供科研

货号：A701042 标准纯度： {[allProObj[0].p_purity_real_show]}
批次查询： 批次纯度:

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产品名称 Hydroxylase ↓ 其他靶点 纯度
Ro 61-8048 +++

KMO, Ki: 4.8 nM

KMO, IC50: 37 nM

 		99%+
DMOG ✔ 98%
Meldonium ✔ 95%
Nepicastat HCl +++

Human dopamine-beta-hydroxylase, IC50: 9 nM

Bovine dopamine-beta-hydroxylase, IC50: 8.5 nM

 		98%
Telotristat etiprate ✔ 99%+
Osilodrostat ++++

11β-hydroxylase, IC50: 2.5 nM

 		95%
H-DL-Phe(4-Cl)-OH ✔ 95%
1. 鼠标悬停在“+”上可以显示相关IC50的具体数值。"+"越多，抑制作用越强。2. "✔"表示该化合物对相应的亚型有抑制作用，但抑制强度暂时没有相关数据。

Sapropterin 2HCl/盐酸沙丙蝶呤 生物活性

描述 Sapropterin dihydrochloride is a synthetic version of tetrahydrobiopterin, the naturally occurring pterin cofactor that is required for PAH-mediated phenylalanine hydroxylation[3]. It acts as a pharmacological chaperone, reducing blood Phe concentration and increasing dietary Phe tolerance in BH4-responsive patients with PAH (phenylalanine hydroxylase) deficiency[4]. In patients with PKU (phenylketonuria) who are responders, treatment with BH4 can activate residual PAH (Phenylalanine hydroxylase) enzyme, improve the oxidative metabolism of Phe, and decrease Phe blood levels in some patients. Kuvan (sapropterin dihydrochloride) is available as 100 mg oral tablets and the recommended starting dose is 10 mg/kg/day daily[5]. Sapropterin dihydrochloride (BH4) reduces phenylalanine (Phe) levels and improves white matter integrity in a subset of individuals with phenylketonuria (PKU) known as "responders."[6]. When started after disease onset, oral sapropterin treatment inhibits chronic hypoxia-induced pulmonary hypertension at least in part by recoupling eNOS in the pulmonary vasculature of newborn piglets[7].

Sapropterin 2HCl/盐酸沙丙蝶呤 动物实验

Species
Animal Model
Administration Dosage Frequency Description References
Mice Pregestational diabetes mellitus model Oral 10 mg/kg per day Once daily during gestation Sapropterin treatment significantly reduced the incidence of congenital heart defects induced by pregestational diabetes mellitus from 59% to 27%, and improved cell proliferation and endothelial nitric oxide synthase (eNOS) function in the fetal heart. J Am Heart Assoc. 2018 Nov 6;7(21):e009624
Mice Experimental autoimmune encephalomyelitis (EAE) Oral 2 mg/day (~100 mg/kg/day) Once daily, starting at the day of immunization To assess the effects of Sapropterin on the course of EAE. Results showed that Sapropterin-treated mice had higher EAE disease scores associated with higher numbers of T-cells infiltrating the spinal cord, but normal T-cell subpopulations in spleen and blood. Neurotherapeutics. 2021 Jul;18(3):1862-1879
Mice Hyperphenylalaninemic mouse model Oral gavage 20, 40, or 100 mg/kg Once daily for 4 days To evaluate the effect of sapropterin treatment upon monoamine neurotransmitter status in hyperphenylalaninemic mice. Results showed that sapropterin treatment significantly increased brain biopterin content but had no effect on serum or brain phenylalanine concentrations. Additionally, sapropterin treatment increased the metabolites HVA and 5-HIAA of dopamine and serotonin, respectively, but did not alter the absolute amounts of dopamine and serotonin. Mol Genet Metab. 2016 Jan;117(1):5-11

Sapropterin 2HCl/盐酸沙丙蝶呤 临床研究

NCT号 适应症或疾病 临床期 招募状态 预计完成时间 地点
NCT02677870 Phenylketonuria Phase 4 Recruiting August 2018 United States, Ohio ... 展开 >> University Hospitals Cleveland Medical Center Recruiting Cleveland, Ohio, United States, 44106 Contact: Lori P Schillaci, MD    216-844-3936    lori-anne.schillaci@uhhospitals.org 收起 <<
NCT00625820 Kidney Disease ... 展开 >> Albuminuria 收起 << Phase 2 Completed - United States, Michigan ... 展开 >> University of Michigan Ann Arbor, Michigan, United States, 48109 收起 <<
NCT00625820 - Completed - -

Sapropterin 2HCl/盐酸沙丙蝶呤 参考文献

[1]Yildiz Y, Dursun A, et al. Partial hydatidiform mole in a phenylketonuria patient treated with sapropterin dihydrochloride. Gynecol Endocrinol. 2017 Jan;33(1):19-20.

[2]unal o, Gokmen-ozel H, et al. Sapropterin dihydrochloride treatment in Turkish hyperphenylalaninemic patients under age four. Turk J Pediatr. 2015 May-Jun;57(3):213-8.

[3]Harding CO. New era in treatment for phenylketonuria: Pharmacologic therapy with sapropterin dihydrochloride. Biologics. 2010 Aug 9;4:231-6

[4]Muntau AC, Adams DJ, Bélanger-Quintana A, Bushueva TV, Cerone R, Chien YH, Chiesa A, Coşkun T, de Las Heras J, Feillet F, Katz R, Lagler F, Piazzon F, Rohr F, van Spronsen FJ, Vargas P, Wilcox G, Bhattacharya K. International best practice for the evaluation of responsiveness to sapropterin dihydrochloride in patients with phenylketonuria. Mol Genet Metab. 2019 May;127(1):1-11

[5]Clinical Review Report: Sapropterin dihydrochloride (Kuvan) [Internet]. Ottawa (ON): Canadian Agency for Drugs and Technologies in Health; 2017 Sep

[6]Hawks Z, Shimony J, Rutlin J, Grange DK, Christ SE, White DA. Pretreatment cognitive and neural differences between sapropterin dihydrochloride responders and non-responders with phenylketonuria. Mol Genet Metab Rep. 2017 Feb 23;12:8-13

[7]Dikalova A, Aschner JL, Kaplowitz MR, Summar M, Fike CD. Tetrahydrobiopterin oral therapy recouples eNOS and ameliorates chronic hypoxia-induced pulmonary hypertension in newborn pigs. Am J Physiol Lung Cell Mol Physiol. 2016 Oct 1;311(4):L743-L753

Sapropterin 2HCl/盐酸沙丙蝶呤 实验方案

计算器
存储液制备 1mg 5mg 10mg

1 mM

5 mM

10 mM

3.18mL

0.64mL

0.32mL

15.91mL

3.18mL

1.59mL

31.83mL

6.37mL

3.18mL

Sapropterin 2HCl/盐酸沙丙蝶呤 技术信息

CAS号69056-38-8
分子式C9H17Cl2N5O3
分子量 314.17
SMILES Code O=C1N=C(N)NC2=C1N[C@@H]([C@@H](O)[C@@H](O)C)CN2.[H]Cl.[H]Cl
MDL No. MFCD00891665
别名 (6R)-BH4 dihydrochloride; (6R)-Tetrahydro-L-biopterin dihydrochloride; BH4; (6R)-5,6,7,8-tetrahydro-L-Biopterin (hydrochloride); R-THBP Dihydrochloride; Tetrahydrobiopterin Dihydrochloride; Sapropterin dihydrochloride
运输蓝冰
InChI Key RKSUYBCOVNCALL-NTVURLEBSA-N
Pubchem ID 135409471
存储条件

In solvent -20°C: 3-6个月 -80°C: 12个月

Pure form Inert atmosphere, store in freezer, under -20°C
溶解方案

DMSO: 190 mg/mL(604.77 mM)，注意：DMSO长时间开封后，会吸水并导致溶解能力下降，请避免使用长期开封的DMSO

H2O: 100 mg/mL(318.3 mM)，配合低频超声助溶

请根据您的动物给药指南选择适当的溶解方案。
以下溶解方案都请先按照体外实验的方式配制澄清的储备液，再依次添加助溶剂：
——为保证实验结果的可靠性，澄清的储备液可以根据储存条件，适当保存；体内实验的工作液，建议现用现配，当天使用； 以下溶剂前显示的百分比是指该溶剂在终溶液中的体积占比；如在配制过程中出现沉淀、析出现象，可以通过加热和/或超声的方式助溶
方案 一
方案 二
方案 三
配制的工作液建议现用现配，短期内尽快用完。 以下溶剂前显示的百分比是指该溶剂在终溶液中的体积占比；如在配制过程中出现沉淀、析出现象，可以通过加热和/或超声的方式助溶。
方案 一

Tags: Sapropterin | 69056-38-8 | (6R)-BH4 | (6R)-Tetrahydro-L-biopterin | PKU | Natural Products | Disease Research Fields | Cardiovascular Disease | 仅供科研使用 | AmBeed-信号通路专用抑制剂 | Sapropterin 2HCl/盐酸沙丙蝶呤 纯度/质量文件 | Sapropterin 2HCl/盐酸沙丙蝶呤 亚型比较 | Sapropterin 2HCl/盐酸沙丙蝶呤 生物活性 | Sapropterin 2HCl/盐酸沙丙蝶呤 临床数据 | Sapropterin 2HCl/盐酸沙丙蝶呤 参考文献 | Sapropterin 2HCl/盐酸沙丙蝶呤 实验方案 | Sapropterin 2HCl/盐酸沙丙蝶呤 技术信息

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