Chloride Channels in Normal and Cystic Fibrosis Intestinal Epithelia

正常及囊性纖維變性的腸道上皮細胞之氯離子通道

蔡麗敏(Lih-Min Tsai) ； 謝博軒(Po-Shiuan Hsieh)

《Journal of Medical Sciences》 13卷3期 (1992/12) Pp. 149-156

摘要

腸道上皮細胞氯離子通道藉由其他離子運送器的共同運作，而調節水分及電解質的吸收或分泌，而在患有囊性纖維變性此種遺傳疾的病人，其腸子之氯離子通道則無法正常運作。正常情況下，腸道上皮細胞內環腺苷酸或鈣離子濃度的增加，會導致氯離子通道的活化；相對地，囊性纖維變性之腸上皮組織對此兩種物質皆不起反應。近年來，與囊性纖維變性有關的基因已被鑑識、分離出來，且由此基因所轉譯的蛋白質被命名為CFTR。目前已有充分的證據顯示CFTR是一種氯離子通道蛋白質，至於此種基因的病變如何導致電解質運送的不正常，則有待進一步的研究。

關鍵字

氯離子通道 ； 上皮組織 ； 腸 ； 囊性纖維變性

並列摘要

Chloride channels in intestinal epithalia act together with other ion transporters to midiate absorption or secretion of water and electrolytes. In cystic fibrosis (CF) which is a lethal genetic disease, the intestinal CF secretion is impaired. Normally, CF channels can be activated by an increase in intracellular level of cAMP or Ca^(2+). In contrast, the CF intestinal epithelia do not respond to either cAMP- or Ca^(2+)-mediated secretagogues. The gene responsible for CF has recently been identified and the protein it encodes has been termed CF transmembrane conductance regulator (CFTR). Convincing evidence suggests that CFTR is a chloride channel. Further studies needs to elucidate the mechanisms by which this gene defect alters the electrolyte transport.

並列關鍵字

Chloride channel ； Epithelium ； Intestine ； Cystic fibrosis