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首页> 外文期刊>Journal of Theoretical Biology >Model of the cAMP activation of chloride transport by CFTR channel and the mechanism of potentiators.
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Model of the cAMP activation of chloride transport by CFTR channel and the mechanism of potentiators.

机译：CFTR通道激活cAMP激活氯转运的模型及增效剂的机制。

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摘要

Mutations of the cystic fibrosis transmembrane conductance regulator (CFTR) cause cystic fibrosis, a hereditary lethal disease. CFTR is a chloride channel expressed in the apical membrane of epithelia. It is activated by cAMP dependent phosphorylation and gated by the binding of ATP. The impaired chloride transport of some types of cystic fibrosis mutations could be pharmacologically solved by the use of chemical compounds called potentiators. Here it is undertaken the construction of a model of the CFTR activation pathways, and the possible modification produced by a potentiator application. The model yields a novel mechanism for the potentiator action, describing the activatory and inhibitory activities on two different positions in the CFTR activation pathway.
机译：囊性纤维化跨膜电导调节剂（CFTR）的突变会导致囊性纤维化，这是一种遗传性致死性疾病。 CFTR是上皮顶膜中表达的氯离子通道。它通过依赖cAMP的磷酸化被激活，并通过ATP的结合被门控。某些类型的囊性纤维化突变的氯离子转运受损可通过药理学方法使用称为增效剂的化合物来解决。在此进行CFTR激活途径模型的构建，以及通过增效剂应用产生的可能修饰。该模型产生了一种新型的增效剂机制，描述了CFTR激活途径中两个不同位置的激活和抑制活性。

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