Ben-Yoseph Y, Defranco C L, Nadler H L
Clin Chim Acta. 1979 Nov 15;99(1):31-5. doi: 10.1016/0009-8981(79)90136-0.
Purified preparations of plasma alpha 2-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 alpha 2-macroglobulin in cystic fibrosis, presumably due to a defective posttranslational process.
来自囊性纤维化患者的血浆α2-巨球蛋白纯化制剂显示,其总己糖含量正常,但唾液酸含量降低多达40%。与对照组的正常数值相比,这些制剂与伴刀豆球蛋白A和麦胚凝集素的结合明显减少。在 obligate 杂合子中发现了中间值。这些结果表明,囊性纤维化患者α2-巨球蛋白的碳水化合物部分可能发生了改变,推测是由于翻译后过程存在缺陷。