Crawford I, Maloney P C, Zeitlin P L, Guggino W B, Hyde S C, Turley H, Gatter K C, Harris A, Higgins C F
Department of Physiology Johns Hopkins University School of Medicine, Baltimore, MD 21205.
Proc Natl Acad Sci U S A. 1991 Oct 15;88(20):9262-6. doi: 10.1073/pnas.88.20.9262.
Antisera against two peptides, corresponding to different domains of the cystic fibrosis gene product CFTR, have been raised and extensively characterized. Both antisera recognize CFTR as a 165-kDa polypeptide in Western analysis of cells transfected with CFTR cDNA as well as in epithelial cell lines. The cell and tissue distribution of CFTR has been studied by immunocytochemistry. CFTR is abundant in epithelial cells, including those lining sweat ducts, small pancreatic ducts, and intestinal crypts. Unexpectedly, the level of CFTR in lung epithelia is relatively low, while it is abundant in the epithelia of kidney tubules. The protein appears to be restricted to the apical, rather than basolateral, regions of epithelial cells and at least a proportion is associated with the plasma membrane. The cell and tissue distributions of CFTR are consistent with a function for this protein as a chloride channel or as a regulator of channel activity.
针对与囊性纤维化基因产物CFTR不同结构域相对应的两种肽段制备了抗血清，并对其进行了广泛的表征。在对用CFTR cDNA转染的细胞以及上皮细胞系进行的Western分析中，两种抗血清均将CFTR识别为一种165 kDa的多肽。通过免疫细胞化学研究了CFTR在细胞和组织中的分布。CFTR在上皮细胞中含量丰富，包括那些衬于汗腺导管、胰腺小导管和肠隐窝的上皮细胞。出乎意料的是，肺上皮细胞中CFTR的水平相对较低，而在肾小管上皮细胞中含量丰富。该蛋白似乎局限于上皮细胞的顶端区域，而非基底外侧区域，并且至少有一部分与质膜相关。CFTR在细胞和组织中的分布与该蛋白作为氯离子通道或通道活性调节剂的功能一致。
