来自囊性纤维化患者的培养上皮细胞中，14 kDa钙结合蛋白CFA的表达增加。

Cultured epithelial cells from patients with cystic fibrosis have an increased expression of the 14 kDa Ca2(+)-binding protein CFA.

作者信息

Keulemans J, Van Heyningen V, Scholte B J, Bijman J, van der Kamp A W, Kansen M, De Jonge H, Galjaard H, Hoogeveen A T

机构信息

MGC Department of Cell Biology and Genetics, Erasmus University, Rotterdam, The Netherlands.

出版信息

Biochem Biophys Res Commun. 1991 Feb 14;174(3):1281-6. doi: 10.1016/0006-291x(91)91560-y.

DOI:10.1016/0006-291x(91)91560-y

PMID:1996991

Abstract

The Cystic Fibrosis antigen (CFA) is a 14 kDa. Ca2(+)-binding protein known to be expressed in cells of myeloid origin during normal cell differentiation. CFA serum levels are elevated in Cystic Fibrosis (CF) patients and heterozygotes. We examined the expression of CFA in different cultured epithelial cells from controls and patients with CF. The steady state level of CFA was in general higher in epithelial cells from CF patients compared to control cells and was found to increase during cell aging. The latter difference could be attributed to an increased rate of CFA synthesis rather than to an impairment of CFA degradation or secretion, as shown by pulse chase experiments.

摘要

囊性纤维化抗原（CFA）是一种14千道尔顿的钙结合蛋白，已知在正常细胞分化过程中于髓系来源的细胞中表达。囊性纤维化（CF）患者及杂合子的CFA血清水平升高。我们检测了来自对照者和CF患者的不同培养上皮细胞中CFA的表达。与对照细胞相比，CF患者上皮细胞中CFA的稳态水平总体上更高，且在细胞衰老过程中升高。脉冲追踪实验表明，后一种差异可归因于CFA合成速率的增加，而非CFA降解或分泌受损。