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先天性纤维蛋白稳定因子缺乏症患者血浆α和β亚基的电免疫测定

Electroimmunoassay of plasma subunits-a and -s in a case of congenital fibrin stabilizing factor deficiency.

作者信息

Barbui T, Cartei G, Chisesi T, Dini E

出版信息

Thromb Diath Haemorrh. 1974 Sep 30;32(1):124-31.

PMID:4454035
Abstract

Antigenic material related to the fibrin stabilizing factor (Factor XIII-FSF) has been found in all the examined patients with congenital deficiency, albeit in reduced amount. Recently, Bohn et al. (1973) showed that plasma Factor-XIII is composed by two subunits, A (or a) and S (or b); the former active protein, the latter probably FSF-vinding protein. Using specific anti-S and anti-A antisera the Authors found, in a case of congenital deficiency of factor XIII, absence of subunit A. Subunit S presented abnormal behaviour as demonstrated by means of quantitative immunoelectrophoresis according to Laurell.

摘要

在所有接受检查的先天性缺乏症患者中均发现了与纤维蛋白稳定因子(因子 XIII - FSF)相关的抗原物质,尽管其含量有所减少。最近,博恩等人(1973 年)表明,血浆因子 XIII 由两个亚基组成,即 A(或 a)和 S(或 b);前者是活性蛋白,后者可能是 FSF 结合蛋白。作者使用特异性抗 S 和抗 A 抗血清,在一例因子 XIII 先天性缺乏症患者中发现亚基 A 缺失。根据劳雷尔的方法进行定量免疫电泳显示,亚基 S 表现出异常行为。

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