Chamson A, Claudy A, Frey J
Acta Derm Venereol. 1987;67(1):16-23.
Collagen metabolism was studied in fibroblast cultures from a patient presenting an epidermolysis bullosa dystrophica recessiva (EBDR) syndrome characterized, in particular, by blistering below the basal lamina observed by electron microscopy. The previously described increase in collagen production was confirmed and several other qualitative modifications of the secreted collagen were observed, including an underhydroxylation of lysine, a decrease in the type III/type I collagen ratio, and an increase in the rapidly degraded collagen. On the other hand, these fibroblasts were able to organize and contract collagen to form a dermal equivalent like normal fibroblasts. Normal keratinocytes can grow and form an epidermal sheet on the surface of these dermal equivalents including normal or pathological fibroblasts.
对一名患有隐性营养不良性大疱性表皮松解症（EBDR）综合征患者的成纤维细胞培养物中的胶原蛋白代谢进行了研究，该综合征的特征尤其在于电子显微镜观察到基底层以下出现水疱。之前描述的胶原蛋白产量增加得到了证实，并且观察到分泌的胶原蛋白的其他几种定性修饰，包括赖氨酸羟化不足、III型/ I型胶原蛋白比例降低以及快速降解的胶原蛋白增加。另一方面，这些成纤维细胞能够像正常成纤维细胞一样组织和收缩胶原蛋白以形成真皮替代物。正常角质形成细胞可以在这些包含正常或病理成纤维细胞的真皮替代物表面生长并形成表皮层。
