Cuspin-1
(Synonyms: Chemical Upregulator of SMN Protein-1) 目录号 : GC43332An upregulator of SMN protein
Cas No.:337932-29-3
Sample solution is provided at 25 µL, 10mM.
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The Survival of Motor Neurons (SMN) protein participates in RNA splicing. Decreases in SMN, typically a consequence of defects in the smn1 gene, result in the death of motor neurons and lead to the neurodegenerative disease, spinal muscular atrophy (SMA). Cuspin-1 is a small molecule upregulator of SMN that has been shown in vitro to increase levels of SMN in SMA patient fibroblasts by 50% at 18 µM. Its mechanism of action is thought to involve increased phosphorylation of ERK to initiate Ras-Raf-MEK signaling, which results in an increased rate of SMN translation.
Cas No. | 337932-29-3 | SDF | |
别名 | Chemical Upregulator of SMN Protein-1 | ||
Canonical SMILES | CC1=CC=C(C(C2=CC(Br)=CN=C2)=O)C=C1 | ||
分子式 | C13H10BrNO | 分子量 | 276.1 |
溶解度 | DMF: 25 mg/mL,DMF:PBS(pH 7.2)(1:1): 0.5 mg/ml,DMSO: 16 mg/mL,Ethanol: 1 mg/mL | 储存条件 | Store at -20°C |
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1 mg | 5 mg | 10 mg | |
1 mM | 3.6219 mL | 18.1094 mL | 36.2188 mL |
5 mM | 0.7244 mL | 3.6219 mL | 7.2438 mL |
10 mM | 0.3622 mL | 1.8109 mL | 3.6219 mL |
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% DMSO % % Tween 80 % saline | ||||||||||
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2.
一定要按照顺序依次将溶剂加入,进行下一步操作之前必须保证上一步操作得到的是澄清的溶液,可采用涡旋、超声或水浴加热等物理方法助溶。
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Small molecule screen reveals regulation of survival motor neuron protein abundance by Ras proteins
ACS Chem Biol 2013 May 17;8(5):914-22.PMID:23496866DOI:PMC3665055
Small molecule modulators of protein activity have proven invaluable in the study of protein function and regulation. While inhibitors of protein activity are relatively common, small molecules that can increase protein abundance are rare. Small molecule protein upregulators with targeted activities would be of value in the study of the mechanisms underlying loss-of-function diseases. We developed a high-throughput screening approach to identify small molecule upregulators of the Survival of Motor Neuron protein (SMN), whose decreased levels cause the neurodegenerative disease spinal muscular atrophy (SMA). We screened 69,189 compounds for SMN upregulators and performed mechanistic studies on the most active compound, a bromobenzophenone analogue designated Cuspin-1. Mechanistic studies of Cuspin-1 revealed that increasing Ras signaling upregulates SMN protein abundance via an increase in translation rate. These findings suggest that controlled modulation of the Ras signaling pathway may benefit patients with SMA.