CFTR
CFTR is a plasma membrane protein belonging to the superfamily of ABC transporters. CFTR works as an ion channel permeable to Cl-, bicarbonate, thiocyanate, and other anions. CFTR structure includes: transmembrane helices that form the conductive pathway and probably the gate; two NBDs, NBD1, and NBD2 that face the cytosolic side and dimerize by binding to two molecules of intracellular ATP; a cytosolic region, the R domain, not present in other ABC proteins, that is the site of cAMP-dependent phosphorylation. Intracellular cAMP elevation is the trigger for CFTR activation. Activation of CFTR requires phosphorylation by protein kinase A (PKA) at multiple sites located at the regulatory domain (RD). Because channel phosphorylation by PKA is mandatory for channel activity, CFTR channel is frequently defined as a ‘‘cAMP-activated channel’’. Protein kinase-C (PKC) dependent phosphorylation at multiple sites is necessary for complete PKA-dependent activation of CFTRLoss of CFTR function is the cause of the multiorgan defects that characterize cystic fibrosis (CF), a genetic disease due to CFTR gene mutations. CF manifestations include: progressive loss of respiratory function, pancreatic insufficiency, biliary cirrhosis, male infertility, and excessive salt loss by sweating.
References
1.Zegarra-Moran O,et al. Cell Mol Life Sci. 2017 Jan;74(1):117-128.
2.Moran O,et al. Cell Mol Life Sci. 2017 Jan;74(1):85-92.
References
1.Zegarra-Moran O,et al. Cell Mol Life Sci. 2017 Jan;74(1):117-128.
2.Moran O,et al. Cell Mol Life Sci. 2017 Jan;74(1):85-92.
Apoptosis
CFTR
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CFTR corrector 12
产品货号 : M37242
cas no: 958941-60-1
CFTR corrector 12 (compound 17C) 是一种双噻唑衍生物,用作CFTR校正剂。CFTR corrector 12 能够校正一些负责控制氯离子跨质膜运输的通道的折叠缺陷突变。CFTR corrector 12 恢复突变细胞中 α-肌聚糖 (α-SG) 含量。 -
WAY-326766
产品货号 : M36981
cas no: 853138-67-7
WAY-326766 increases ion transport through mutant CFTR and can be used to alter the lifespan of eukaryotes.WAY-326766 can be used to treat cystic fibrosis (CF). -
CFTR corrector 8
产品货号 : M35744
cas no: 1918142-35-4
CFTR corrector 8 是一种有效的 CFTR 调制器。CFTR 可用于囊性纤维化的研究。 -
SRI-37240
产品货号 : M35584
cas no: 883956-47-6
SRI-37240是一种有效的提前终止密码子 (PTCs) 抑制剂。SRI-37240 抑制 CFTR 的无义突变。在 HEK293T 细胞中,SRI-37240 改变 PTCs 的细胞翻译终止。当与 G418 使用联合时,SRI-37240 还能恢复原发性支气管上皮细胞的 CFTR 功能。 -
CFTR corrector 9
产品货号 : M35450
cas no: 909861-78-5
CFTR corrector 9 (compound 42) 是一种囊性纤维化跨膜传导调节蛋白 (CFTR) 调节剂。CFTR corrector 9 可用于囊性纤维化 (CF) 和其他 CFTR 相关疾病的研究。