Oakley C A, Priestley G C
Acta Derm Venereol. 1985;65(4):277-81.
Collagen synthesis was measured in fibroblasts cultured from the skin of six patients with epidermolysis bullosa simplex, from six patients with dystrophic epidermolysis bullosa and six age-matched controls without skin disease. Both groups of patients' fibroblasts synthesized approximately twice as much collagen (dpm/cell) as the controls. Synthesis of other proteins showed a smaller increase. Collagenase activity in culture media from four fibroblast lines per group was measured, using a 3H-collagen substrate, both before and after trypsin treatment to activate procollagenase. As expected, the dystrophic group had the highest activity (30% more than controls) and the result was little affected by trypsin: the enzyme appeared to be in active form. Enzyme activity in the simplex group was increased from 67% to 114% of control values by trypsin treatment. The excessive collagen synthesis in both dystrophic and simplex fibroblasts may be a consequence of their greater collagenase activity and suggests an unsuspected dermal involvement in epidermolysis bullosa simplex. Our data confirm an excessive secretion of collagenase by dystrophic fibroblasts but suggest that the enzyme's state of activation may be the important aetiological feature of the dystrophic disease.
在取自6例单纯性大疱性表皮松解症患者皮肤、6例营养不良性大疱性表皮松解症患者皮肤以及6例无皮肤疾病的年龄匹配对照者皮肤的成纤维细胞中检测了胶原蛋白合成。两组患者的成纤维细胞合成的胶原蛋白（每分钟衰变数/细胞）均约为对照者的两倍。其他蛋白质的合成增加幅度较小。使用3H - 胶原蛋白底物，在胰蛋白酶处理激活前胶原酶前后，对每组4条成纤维细胞系培养基中的胶原酶活性进行了测定。正如预期的那样，营养不良组的活性最高（比对照者高30%），且该结果受胰蛋白酶影响较小：该酶似乎处于活性形式。单纯性大疱性表皮松解症组经胰蛋白酶处理后，酶活性从对照值的67%增加到114%。营养不良性和单纯性大疱性表皮松解症成纤维细胞中胶原蛋白合成过多可能是其胶原酶活性较高的结果，这表明单纯性大疱性表皮松解症存在未被怀疑的真皮受累情况。我们的数据证实了营养不良性成纤维细胞胶原酶分泌过多，但表明该酶的激活状态可能是营养不良性疾病重要的病因学特征。
