Rao G J, Spells G, Nadler H L
Pediatr Res. 1977 Sep;11(9 Pt 1):981-5. doi: 10.1203/00006450-197709000-00010.
Homogenates of cultivated skin fibroblasts derived from patients with cystic fibrosis had a higher level of UDP-galactose:ovalbumin galactosyl transferase activity compared to fibroblasts derived from control subjects. The activity in control subjects was 1.82 +/- 0.43 nmol galactose transferred/hr/mg protein, whereas the activity in fibroblasts of patients was 2.95 +/- 0.77. The difference was significant at P less than 0.01. Activity in the fibroblasts of obligate heterozygotes was 2.15 +/- 0.60. The difference between the activities in fibroblasts of heterozygotes and patients was significant at P less than 0.05. The activity in control fibroblasts could be enhanced by basic polypeptides like polylysine, polyarginine, histone, and protamine but not by neutral or acidic polypeptides. Fibroblasts from patients released significantly higher amounts of a soluble form of the enzyme activity into the culture medium than control fibroblasts.
与来自对照受试者的成纤维细胞相比，囊性纤维化患者培养的皮肤成纤维细胞匀浆具有更高水平的UDP-半乳糖：卵清蛋白半乳糖基转移酶活性。对照受试者中的活性为1.82±0.43 nmol半乳糖转移/小时/毫克蛋白质，而患者成纤维细胞中的活性为2.95±0.77。差异在P小于0.01时具有统计学意义。 obligate杂合子的成纤维细胞中的活性为2.15±0.60。杂合子和成纤维细胞患者中的活性差异在P小于0.05时具有统计学意义。对照成纤维细胞中的活性可通过碱性多肽如聚赖氨酸、聚精氨酸、组蛋白和鱼精蛋白增强，但不能通过中性或酸性多肽增强。与对照成纤维细胞相比，来自患者的成纤维细胞向培养基中释放的可溶性酶活性形式的量明显更高。
