Margolies R, Boat T F
Pediatr Res. 1983 Dec;17(12):931-5. doi: 10.1203/00006450-198312000-00001.
We compare the complete carbohydrate composition of IgG purified from the serum of patients with cystic fibrosis (CF) with that of control subjects. Our results indicate that IgG from cystic fibrosis patients is underglycosylated with respect to galactose and sialic acid, the two terminal sugars on the antennae of the complex-type oligosaccharide chains found on IgG. The galactose content, as determined by gas liquid chromatography, was 2.81 +/- 0.86 (S.D.) mole/mole of IgG for normal subjects versus 1.5 +/- 0.39 for CF subjects (P less than 0.025). Sialic acid content, as determined by the Warren procedure, was 1.33 +/- 0.39 for normals versus 0.47 +/- 0.10 for CF subjects (P less than 0.001). Neither galactose nor sialic acid values for the two groups overlapped. The contents of the core sugars, mannose and glucosamine, and of fucose were not significantly different. When the data are expressed as residues per 3 moles mannose, similar results are obtained. We suggest that immune complex formation, which has been documented in many CF patients, exposes sugar chains of IgG molecules to hydrolytic activity of serum glucosidases, resulting in partial removal of the more peripheral sugars. Because serum glycoproteins missing sialic acid and galactose are not readily cleared from the circulation, the observed changes may contribute to elevated levels of IgG and immune complexes in older people with CF.
我们比较了从囊性纤维化(CF)患者血清中纯化的IgG与对照受试者的完整碳水化合物组成。我们的结果表明,CF患者的IgG在半乳糖和唾液酸方面存在糖基化不足,这两种糖是在IgG上发现的复合型寡糖链触角上的两个末端糖。通过气相色谱法测定,正常受试者的半乳糖含量为每摩尔IgG 2.81±0.86(标准差)摩尔,而CF受试者为1.5±0.39(P<0.025)。通过沃伦方法测定,正常受试者的唾液酸含量为1.33±0.39,而CF受试者为0.47±0.10(P<0.001)。两组的半乳糖和唾液酸值均无重叠。核心糖、甘露糖和氨基葡萄糖以及岩藻糖的含量没有显著差异。当数据表示为每3摩尔甘露糖的残基时,得到了类似的结果。我们认为,许多CF患者中已记录的免疫复合物形成使IgG分子的糖链暴露于血清糖苷酶的水解活性中,导致更多外周糖的部分去除。由于缺乏唾液酸和半乳糖的血清糖蛋白不易从循环中清除,观察到的变化可能导致老年CF患者中IgG和免疫复合物水平升高。