- 中文名称
Ataluren | Translarna™；3- [5-（2-氟苯基）-1,2,4-恶二唑-3-基]-苯甲酸
- 英文名字
- Ataluren|Translarna™; 3-[5-(2-fluorophenyl)-1,2,4-oxadiazol-3-yl]-benzoic acid
- 供应商
- Cayman Chemical
- 产品货号
- 16758-10
- 产品报价
- ￥询价/10mg
- 产品说明书
- 点击查看
- 购买方式
- 银行转账、电汇、支票、现金，在线支付宝及网银支付，或直接与我们电话联系400-6800-868
- 产品新闻
- 背景资料
- Nonsense mutations create a premature termination of mRNA translation and have been implicated in various genetic disorders, including muscular dystrophy and cystic fibrosis.{27189} PTC-124 is a nonaminoglycoside that has been reported to selectively induce ribosomes to read through premature nonsense stop signals on mRNA, thus allowing the production of full-length, functional proteins.{27189} In a mouse model of cystic fibrosis caused by nonsense mutations, PTC-124 treatment (60 mg/kg s.c. injection or 0.3-0.9 mg/ml orally) has been shown to restore cystic fibrosis transmembrane conductance regulator (CFTR) protein expression and function.{27187} The target activity of PTC-124 was initially evaluated by firefly luciferase reporter cell-based nonsense codon assay (IC50 = 7 nM); however, subsequent assessments using a Renilla reniformis luciferase reporter have failed to produce nonsense codon suppression activity.{27188} Thus, while PTC-124 is in clinical testing in patients with nonsense mutations within the CFTR or dystrophin genes, controversy surrounds its exact mechanism of action.{27189,27188,27191,27190}
- 产品描述
- A nonaminoglycoside that has been reported to selectively induce ribosomes to read through premature nonsense stop signals on mRNA, thus allowing the production of full-length, functional proteins (IC50 = 7 nM); 60 mg/kg s.c. injection or 0.3-0.9 mg/ml orally has been shown to restore CFTR protein expression and function in a mouse model of cystic fibrosis caused by nonsense mutations
- 产品形式
- A crystalline solid
- 保存建议
- 建议您在收到Cayman Chemical品牌的PTC-124 (10 mg)产品后置于-20度保存，产品有效期为730天.
- 其他
- Cayman Chemical向全球科学工作者提供多研究领域的生化、免疫试剂和分析试剂盒，其产品被广泛应用于肿瘤、氧化氮、神经学、凋亡、氧化性损伤、内分泌学等不同研究领域.Cayman Chemical可提供用于检测的特色试剂盒，如：类花生酸类物质、游离的生物标志、环核苷、激素及氧化氮等；也提供多种高质量试剂，包括：类花生酸类物质、氧化氮试剂及许多相关脂质、脂肪酸、酶和抗体.作为Cayman Chemical在中国的区域总代理，艾美捷科技有限公司将为中国客户提供最全面的Cayman Chemical产品以及客户订制化服务.
- 注意
-
该页面的中文产品信息的翻译，仅供参考。准确的产品信息请以厂家的英文说明书为准。下单前，请浏览说明书确认。
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