Guggino W B
Johns Hopkins University School of Medicine, Baltimore 21205.
J Bioenerg Biomembr. 1993 Feb;25(1):27-35. doi: 10.1007/BF00768065.
Outwardly rectifying Cl- channels were originally thought to be the central element in cystic fibrosis. The role of these channels in CF was questioned to such an extent that doubts were raised about the validity of the original experiments. Recent data reestablishes a role for outwardly rectifying Cl- channels (ORCC) in CF and suggests that the protein encoded by the CF gene, the cystic fibrosis transmembrane regulator (CFTR), can effect the regulation of more than one channel in the airway. This minireview deals with the rise, fall, and resurrection of the role of outwardly rectifying Cl- channels in CF.
外向整流性氯离子通道最初被认为是囊性纤维化的核心要素。这些通道在囊性纤维化中的作用受到了极大质疑,以至于人们对最初实验的有效性产生了怀疑。最近的数据重新确立了外向整流性氯离子通道(ORCC)在囊性纤维化中的作用,并表明囊性纤维化基因编码的蛋白质,即囊性纤维化跨膜传导调节因子(CFTR),可以影响气道中不止一种通道的调节。这篇小型综述探讨了外向整流性氯离子通道在囊性纤维化中作用的兴起、衰落和复兴。