Park C M, Reid P E, Applegarth D A, Wong L T, MacDonald I B
Pediatr Res. 1985 Apr;19(4):344-6. doi: 10.1203/00006450-198519040-00005.
The putative involvement of alpha 2-macroglobulin (alpha 2M) in the pathogenesis of cystic fibrosis has long been a subject of controversy. Previous reports have indicated that there are alterations in the carbohydrate moieties of alpha 2M in the disease. In the present study the carbohydrate composition of alpha 2M isolated from the plasma of six patients with CF was compared to that of alpha 2M from six age- and sex-matched normal controls. The carbohydrate composition of CF alpha 2M expressed as mean mumol carbohydrate +/- SD per 100 mg protein was: fucose 0.70 +/- 0.12; mannose 14.07 +/- 1.31; galactose 6.72 +/- 0.65; glucosamine 15.38 +/- 1.59; sialic acid 5.52 +/- 0.33 while that of normal control alpha 2M was: fucose 0.69 +/- 0.11; mannose 14.42 +/- 1.21; galactose 6.91 +/- 0.52; glucosamine 16.13 +/- 1.77; sialic acid 5.58 +/- 0.31. Therefore, contrary to previous reports, this paper demonstrates that there is no difference in the carbohydrate composition of alpha 2M in cystic fibrosis.
α2巨球蛋白（α2M）假定参与囊性纤维化的发病机制长期以来一直是一个有争议的话题。先前的报告表明，该疾病中α2M的碳水化合物部分存在改变。在本研究中，将从6名囊性纤维化患者血浆中分离出的α2M的碳水化合物组成与6名年龄和性别匹配的正常对照者的α2M的碳水化合物组成进行了比较。以每100毫克蛋白质中平均微摩尔碳水化合物±标准差表示的囊性纤维化α2M的碳水化合物组成如下：岩藻糖0.70±0.12；甘露糖14.07±1.31；半乳糖6.72±0.65；葡糖胺15.38±1.59；唾液酸5.52±0.33，而正常对照α2M的碳水化合物组成如下：岩藻糖0.69±0.11；甘露糖14.42±1.21；半乳糖6.91±0.52；葡糖胺16.13±1.77；唾液酸5.58±0.31。因此，与先前的报告相反，本文证明囊性纤维化中α2M的碳水化合物组成没有差异。
