DECREASED SIALIC-ACID AND ALTERED BINDING TO LECTINS OF PURIFIED ALPHA-2-MACROGLOBULIN FROM PATIENTS WITH CYSTIC-FIBROSIS

被引：32

作者：

BENYOSEPH, Y

DEFRANCO, CL

NADLER, HL

机构：

[1] Department of Pediatrics, Northwestern University Medical School, Division of Genetics, Chicago

来源：

CLINICA CHIMICA ACTA | 1979年 / 99卷 / 01期

基金：

美国国家卫生研究院;

关键词：

D O I：

10.1016/0009-8981(79)90136-0

中图分类号：

R446 [实验室诊断]; R-33 [实验医学、医学实验];

学科分类号：

1001 ;

摘要：

Purified preparations of plasma α2-macroglobulin from patients with cystic fibrosis are shown to have normal amounts of total hexose but as much as 40% decrease in their sialic acid content. The binding of these preparations to concanavalin A and wheat-germ agglutinin was markedly reduced as compared to normal values in controls. Intermediate values were found in obligate heterozygotes. These results suggest a possible alteration in the carbohydrate moiety of α2-macroglobulin in cystic fibrosis, presumably due to a defective posttranslational process. © 1979.

引用

页码：31 / 35

页数：5

共 17 条

[1]

ALHADEFF JA, 1978, CLIN GENET, V13, P417

[2]

ALHADEFF JA, 1978, CLIN GENET, V14, P189

[3]

DUNN JT, 1967, J BIOL CHEM, V242, P5549

[4]

Goldstein I J, 1978, Adv Carbohydr Chem Biochem, V35, P127