Leblanc R, Lozano A M, van der Rest M, Guttmann R D
Department of Neurosurgery, Montreal Neurological Hospital, Quebec, Canada.
J Neurosurg. 1989 Jun;70(6):837-40. doi: 10.3171/jns.1989.70.6.0837.
It has been suggested that a deficiency in the expression of type III collagen may play a role in the pathogenesis of cerebral aneurysms. To test this hypothesis in cases of familial cerebral aneurysms, fibroblast cell cultures were established and the expression of collagen types I and III was studied in a patient with three cerebral aneurysms whose mother and sister also had cerebral aneurysms. Cultured skin fibroblasts were labeled with tritiated proline. The collagens and procollagens were precipitated and run on sodium dodecyl sulfate-polyacrylamide gel electrophoresis after reduction to analyze procollagen chains. Control cell lines were analyzed simultaneously. Quantitation of the ratios of type III to type I procollagen synthesis was achieved by integration of the intensities of the pro-alpha 1(III), pro-alpha 1(I), and pro-alpha 2(I) bands on fluorograms of electrophoretic gels of medium proteins. There was no difference in type I and III procollagen levels observed between the cells from the aneurysm patient and those from the control cell lines. These data do not support the hypothesis that familial cerebral aneurysms are caused by a deficiency of type III collagen.
有人提出,III型胶原蛋白表达不足可能在脑动脉瘤的发病机制中起作用。为了在家族性脑动脉瘤病例中验证这一假设,建立了成纤维细胞培养物,并对一名患有三个脑动脉瘤且其母亲和姐姐也患有脑动脉瘤的患者的I型和III型胶原蛋白表达进行了研究。用氚标记的脯氨酸标记培养的皮肤成纤维细胞。将胶原蛋白和前胶原蛋白沉淀,还原后在十二烷基硫酸钠-聚丙烯酰胺凝胶电泳上进行分析以分析前胶原链。同时分析对照细胞系。通过对培养基蛋白电泳凝胶荧光图上的前α1(III)、前α1(I)和前α2(I)条带强度进行积分,实现了III型与I型前胶原合成比率的定量。在动脉瘤患者的细胞和对照细胞系的细胞之间,未观察到I型和III型前胶原水平的差异。这些数据不支持家族性脑动脉瘤是由III型胶原蛋白缺乏引起的这一假设。