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囊性纤维化:成纤维细胞培养基的分级分离显示纤毛抑制。

Cystic fibrosis: fractionation of fibroblast media demonstrating ciliary inhibition.

作者信息

Bowman B H, Barnett D R, Matalon R, Danes B S, Bearn A G

出版信息

Proc Natl Acad Sci U S A. 1973 Feb;70(2):548-51. doi: 10.1073/pnas.70.2.548.

Abstract

A chromatographic fraction has been identified in media from fibroblast cultures derived from 15 of 18 cystic fibrosis patients and 8 of 10 heterozygotes that inhibits ciliary activity, as judged by the oyster gill assay. The analogous fraction of media from cultures of fibroblasts derived from 11 normal individuals did not inhibit ciliary activity under the experimental conditions used. The chromatographic fractions containing the ciliary inhibitory activity from both fibroblast media and sera of cystic fibrosis patients and heterozygotes eluted in the same position from diethylaminoethyl-cellulose at pH 8.5. Although the serum ciliary inhibitor had consistently been detected in a fraction that contained predominantly immunoglobulin G, results of the fractionation of fibroblast media demonstrate that the ciliary inhibitor is present in a protein fraction containing three species of different molecular weight and in which IgG cannot be detected by the techniques used.

摘要

通过牡蛎鳃试验判断,在来自18名囊性纤维化患者中15名患者以及10名杂合子中8名患者的成纤维细胞培养物的培养基中,已鉴定出一种抑制纤毛活动的色谱级分。在所用实验条件下,来自11名正常个体的成纤维细胞培养物的类似培养基级分不抑制纤毛活动。来自囊性纤维化患者和杂合子的成纤维细胞培养基及血清中含有纤毛抑制活性的色谱级分,在pH 8.5条件下从二乙氨基乙基纤维素上以相同位置洗脱。尽管在主要含有免疫球蛋白G的级分中一直检测到血清纤毛抑制剂,但成纤维细胞培养基分级分离的结果表明,纤毛抑制剂存在于一个含有三种不同分子量物质的蛋白质级分中,且用所用技术无法检测到IgG。

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Cystic fibrosis: studies with the oyster ciliary assay.囊性纤维化:牡蛎纤毛试验研究
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Evidence that the ciliary inhibitor of cystic fibrosis is not an antibody.
J Clin Invest. 1973 Nov;52(11):2732-6. doi: 10.1172/JCI107468.
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Cystic fibrosis: current concepts.囊性纤维化:当前概念
J Med Genet. 1974 Sep;11(3):249-52. doi: 10.1136/jmg.11.3.249.

本文引用的文献

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Oyster ciliary inhibition by cystic fibrosis factor.囊性纤维化因子对牡蛎纤毛的抑制作用。
Science. 1969 Apr 18;164(3877):325-6. doi: 10.1126/science.164.3877.325.

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