阿塔鲁伦
| CAS号 | 775304-57-9 | 货号 | BCP01756 |
| 中文名 | 阿塔鲁伦 | ||
| 英文名 | Ataluren | ||
| 中文别名 | |||
| 英文别名 | PTC-124;PTC 124;PTC124; | ||
| 分子式 | C15H9FN2O3 | 分子量 | 284.24 |
| 生物活性 | Compared with Gentamicin which is only active at much higher concentrations, PTC124 is a more potent nonsense-suppressing agent and exhibits 4- to 15-fold stimulation of read-through relative to controls. PTC124 (0.01-3 μM) promotes dose-dependent read-through of all three nonsense codons in HEK293 cells harboring LUC-190 nonsense alleles with the highest read-through at UGA, followed by UAG and then UAA, but it does not suppress multiple proximal nonsense codons. Like Gentamicin, PTC124 is most active when a pyrimidine (in particular cytosine, C) follows the nonsense codon. Consistent with the stable cell line reporter assay, PTC124 (17 μM) promotes significant production of dystrophin in primary muscle cells from Duchenne muscular dystrophy (DMD) patients or MDXMDX mice expressing dystrophin nonsense alleles. PTC124 selectively promotes ribosomal read-through of premature termination but not normal termination codons, even at concentrations substantially greater than the values achie | ||
| 信号通路 | Ion Channel/Membrane Transporter | ||
| 靶 点 | CFTR | ||
结构式
本页面部分数据来自于公开的网络资源,所以瀚香生物不能保证其准确性。
不同批次的产品详情请咨询我们的客服：
- 全国免费电话：400-099-8200
- 在线客服:
瀚香生物（Biochempartner) 的所有产品和服务仅用于科学研究，禁止用于人体和治疗！