Decreased sialic acid and altered binding to lectins of purified alpha 2-macroglobulin from patients with cystic fibrosis.
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.
D003208:Concanavalin A; D003550:Cystic Fibrosis / Q000097:blood* / Q000235:genetics; D006580:Genetic Carrier Screening; D006601:Hexoses / Q000032:analysis; D006801:Humans; D037102:Lectins; D012794:Sialic Acids / Q000032:analysis*; D000511:alpha-Macroglobulins / Q000302:isolation & purification
D006601:Hexoses; D037102:Lectins; D012794:Sialic Acids; D000511:alpha-Macroglobulins; D003208:Concanavalin A