Chen J H, Schulman H, Gardner P
Department of Medicine, Stanford University, CA 94305.
Science. 1989 Feb 3;243(4891):657-60. doi: 10.1126/science.2464852.
A defect in regulation of a chloride channel appears to be the molecular basis for cystic fibrosis (CF), a common lethal genetic disease. It is shown here that a chloride channel with kinetic and regulatory properties similar to those described for secretory epithelial cells is present in both T and B lymphocyte cell lines. The regulation of the channels by adenosine 3',5'-monophosphate (cAMP)-dependent protein kinase in transformed B cells from CF patients is defective. Thus, lymphocytes may be an accessible source of CF tissue for study of this defect, for cloning of the chloride channel complex, and for diagnosis of the disease.
氯离子通道调节缺陷似乎是囊性纤维化（CF）的分子基础，CF是一种常见的致命性遗传病。本文表明，T和B淋巴细胞系中均存在一种氯离子通道，其动力学和调节特性与分泌上皮细胞中所描述的相似。来自CF患者的转化B细胞中，3',5'-环磷酸腺苷（cAMP）依赖性蛋白激酶对该通道的调节存在缺陷。因此，淋巴细胞可能是用于研究此缺陷、克隆氯离子通道复合物以及诊断该疾病的CF组织的一个可获取来源。
