Branaplam hydrochloride LMI070 hydrochloride; NVS-SM1 hydrochloride|cas:1562338-39-9|西域试剂

Branaplam hydrochloride LMI070 hydrochloride; NVS-SM1 hydrochloride,99.42%

产品编号：Bellancom-19620A| CAS NO：1562338-39-9| 分子式：C₂₂H₂₈ClN₅O₂| 分子量：429.94

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货号	价格	库存与货期
Bellancom-19620A	￥1300.00	杭州北京（现货）
Bellancom-19620A	￥2200.00	杭州北京（现货）
Bellancom-19620A	￥8000.00	杭州北京（现货）
Bellancom-19620A	￥13000.00	杭州北京（现货）

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Branaplam hydrochloride LMI070 hydrochloride; NVS-SM1 hydrochloride

产品介绍

Branaplam (LMI070; NVS-SM1) hydrochloride 是一种口服有效和选择性的 SMN2 拼接调节剂，对 SMN 的 EC₅₀ 为 20 nM。Branaplam hydrochloride 抑制 hERG，IC₅₀ 为 6.3 μM。Branaplam hydrochloride 在严重的脊髓性肌萎缩症 (SMA) 小鼠模型中可提高全长 SMN 蛋白并延长其生存期。

生物活性

Branaplam (LMI070; NVS-SM1) hydrochloride is a highly potent, selective and orally active survival motor neuron-2 (SMN2) splicing modulator with an EC₅₀ of 20 nM for SMN. Branaplam hydrochloride inhibits human-ether-a-go-go-related gene (hERG) with an IC₅₀ of 6.3 μM. Branaplam hydrochloride elevates full-length SMN protein and extends survival in a severe spinal muscular atrophy (SMA) mouse model.

体外研究

Branaplam (LMI070; NVS-SM1) hydrochloride treatment induces changes in the levels of 175 genes in human fibroblasts.

西域 has not independently confirmed the accuracy of these methods. They are for reference only.

体内研究

Branaplam (LMI070; NVS-SM1; 3, 10, 30 mg/kg; oral) hydrochloride produces dose-dependent elevations of SMN2-FL transcript and SMN protein in brain and spinal cord.
Branaplam (1 mg/kg of IV; 3 mg/kg of PO) hydrochloride has a CL of 25 mL/min/kg and an AUC of 3.03 μM•h.
A single Branaplam (oral; 30 mg/kg) hydrochloride results in significant and durable SMN protein elevation in brain for up to 160 hours in C/+ mice.
Branaplam (oral; 0.03, 0.1, 0.3, 1, 3 mg/kg) hydrochloride improves body weight and extendes lifespan in n SMNΔ7 mice.

西域 has not independently confirmed the accuracy of these methods. They are for reference only.

体内研究

西域 has not independently confirmed the accuracy of these methods. They are for reference only.

性状

Solid

溶解性数据

In Vitro:

H₂O : 200 mg/mL (465.18 mM; Need ultrasonic)

配制储备液

浓度溶剂体积质量	1 mg	5 mg	10 mg

1 mM	2.3259 mL	11.6295 mL	23.2591 mL
5 mM	0.4652 mL	2.3259 mL	4.6518 mL
10 mM	0.2326 mL	1.1630 mL	2.3259 mL

请根据产品在不同溶剂中的溶解度选择合适的溶剂配制储备液；一旦配成溶液，请分装保存，避免反复冻融造成的产品失效。
储备液的保存方式和期限：-80°C, 6 months; -20°C, 1 month (sealed storage, away from moisture and light)。-80°C 储存时，请在 6 个月内使用，-20°C 储存时，请在 1 个月内使用。

运输条件

Room temperature in continental US; may vary elsewhere.

储存方式

4°C, sealed storage, away from moisture and light

*In solvent : -80°C, 6 months; -20°C, 1 month (sealed storage, away from moisture and light)

参考文献