Synthesis of hyaluronic acid and collagen in skin fibroblasts cultured from patients with osteogenesis imperfecta.
Synthesis of hyaluronic acid and collagen in skin fibroblasts cultured from patients with osteogenesis imperfecta.
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成骨不全患者培养的皮肤成纤维细胞中透明质酸和胶原蛋白的合成。
DOI：
10.1016/0304-4165(80)90388-8
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发表时间：
1980
期刊：
影响因子：
--
通讯作者：
R. Penttinen
中科院分区：
文献类型：
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作者：
H. Turakainen;H. Larjava;H. Saarni;R. Penttinen
通过用[3 H]脯氨酸标记培养物并用DEAE-和CM-纤维素色谱分离胶原蛋白，研究了来自骨生成障碍患者和年龄匹配对照的皮肤成纤维细胞培养物中胶原和透明质酸的合成，或用[3 H]葡糖胺和[14 C]甘氨酸双标记培养物，并通过DEAE-ELISA从糖蛋白和硫酸化蛋白聚糖中分离放射性透明质酸。纤维素层析还测定了细胞层透明质酸合成酶复合物（透明质酸合成酶）的活性。III型胶原蛋白约为。40-50%的总胶原蛋白在第一个品种和约。25-30%在第二个品种。与对照培养物相比，第三种培养物中III型胶原与总胶原的比例无差异。在DEAE-纤维素色谱图中，3 H-标记的透明质酸的放射性与14 C-标记的蛋白质的放射性进行了比较。患者培养物中的比值范围为9.2-17.3，对照培养物中的比值范围为4.6-8.8。透明质酸合成酶活性在成骨细胞中比对照组高1.3-2.0倍，皮肤成纤维细胞中透明质酸合成的增加与疾病的严重程度相关，但与III型胶原合成的增加无关。
Collagen and hyaluronic acid syntheses were studied in skin fibroblast cultures from patients with osteogenesis imperfecta and age-matched controls by labeling the cultures either with [3H]proline and separating the collagenous proteins with DEAE- and CM-cellulose chromatographies, or double-labeling the cultures with [3H]glucosamine and [14C]glycine and separating radioactive hyaluronic acid from glycoproteins and sulphated proteoglycans by DEAE-cellulose chromatography. The activities of the cell layer hyaluronate synthesizing enzyme complex (hyaluronate synthetase) were also determined.The osteogenesis imperfecta cultures were classified into three variants on the basis of type III collagen synthesis. Type III collagen amounted to approx. 40–50% from total collagen in the first variety and approx. 25–30% in the second variety. No difference was noted in the ratio of type III collagen to total collagen in the third variety in comparison with control cultures.The radioactivities of3H-labeled hyaluronic acid in DEAE-cellulose chromatograms were compared with those of the14C-labeled proteins. The ratios ranged 9.2–17.3 in the cultures from the patients and 4.6–8.8 in the control cultures. Hyaluronate synthetase activities were 1.3–2.0-fold higher in the osteogenesis imperfecta cells than in their controls.Increased hyaluronic acid synthesis in skin fibroblasts correlated with the severity of the disease but not with the increase in type III collagen synthesis.