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正常成年人体内组织中精氨酸酶及精氨酸酶缺乏症患者的免疫学研究。

Immunologic studies of arginase in tissues of normal human adult and arginase-deficient patients.

作者信息

Spector E B, Rice S C, Cederbaum S D

出版信息

Pediatr Res. 1983 Dec;17(12):941-4. doi: 10.1203/00006450-198312000-00003.

DOI:10.1203/00006450-198312000-00003
PMID:6419196
Abstract

Rabbit antibody to human liver arginase was used to examine the immunologic characteristics of arginase in red blood cells (RBC), liver, kidney, brain, and gastrointestinal tract from normal adults and from patients with hyperargininemia. Greater than 90% of the arginase in RBC and liver was precipitated by this antibody whereas only 50% of the arginase in kidney, brain, and gastrointestinal tract reacted with it. Two siblings and a third patient with hyperargininemia were found to have immunoreactive arginase protein in their RBC that was enzymatically inactive. The amount of arginase protein approximated that found in RBC from normal individuals. A kidney biopsy obtained from one of the patients with hyperargininemia had arginase activity 4-5-fold greater than that found in normal kidney biopsy material. Double immunodiffusion and precipitation-inhibition studies demonstrated two types of arginase protein in this patient's kidney: one enzymatically inactive and precipitated by the antibody, and one enzymatically active but not precipitated by the antibody. These data, in conjunction with biochemical data reported previously demonstrate that there are two gene loci determining arginase in man.

摘要

用人肝精氨酸酶的兔抗体来检测正常成年人以及高精氨酸血症患者红细胞(RBC)、肝脏、肾脏、大脑和胃肠道中精氨酸酶的免疫学特性。该抗体可沉淀RBC和肝脏中超过90%的精氨酸酶,而肾脏、大脑和胃肠道中只有50%的精氨酸酶与之反应。发现两名患有高精氨酸血症的同胞和另一名患者的RBC中存在免疫反应性精氨酸酶蛋白,但该蛋白无酶活性。精氨酸酶蛋白的量与正常个体RBC中的量相近。从一名高精氨酸血症患者获取的肾脏活检组织中,精氨酸酶活性比正常肾脏活检材料中的高4至5倍。双向免疫扩散和沉淀抑制研究表明,该患者的肾脏中有两种类型的精氨酸酶蛋白:一种无酶活性且可被抗体沉淀,另一种有酶活性但不能被抗体沉淀。这些数据与先前报道的生化数据一起表明,人类中存在两个决定精氨酸酶的基因位点。

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