Gayraud B, Höpfner B, Jassim A, Aumailley M, Bruckner-Tuderman L
Institut de Biologie et Chimie des Protéines, CNRS, 69367 Lyon Cedex 07, France.
J Biol Chem. 1997 Apr 4;272(14):9531-8. doi: 10.1074/jbc.272.14.9531.
Using the monoclonal antibody GDA-J/F3, a 50-kDa noncollagenous component of human skin basement membrane zone was identified. Immunofluorescence stainings of normal human skin with the GDA-J/F3 antibody showed a linear fluorescence decorating the basement membrane zone. With immunoelectron microscopy, the epitope was localized to the insertion points of the anchoring fibrils into the lamina densa. The antigen is distinct from collagen VII, from the main structural protein of the anchoring fibrils, and from several other structural molecules of the basement membrane zone, because the GDA-J/F3 antibody did not react with purified basement membrane components in vitro. In serum-free cultures, the antigen was synthesized and secreted by normal and transformed human keratinocytes and to a lesser extent by normal human skin fibroblasts. Immunoprecipitation of radiolabeled epithelial cell-conditioned medium with the GDA-J/F3 antibody yielded two polypeptides that migrated on SDS-polyacrylamide gel electrophoresis with apparent molecular masses of 46 and 50 kDa under nonreducing conditions. Using reducing gels, only the 50-kDa polypeptide was observed. The antigen was resistant to digestion with bacterial collagenase but sensitive to trypsin and pepsin. It also bound to heparin and DEAE cellulose at low ionic strength and alkaline pH. These findings indicate that the GDA-J/F3 antigen is a small globular disulphide-bonded protein with a potential to interact with basement membrane proteoglycans. Integration of the GDA-J/F3 antigen into the histoarchitecture of the dermo-epidermal junction is dependent on the presence of collagen VII, because the GDA-J/F3 epitope was missing in several patients with a genetic blistering disorder of the skin, epidermolysis bullosa dystrophica, who lacked collagen VII and anchoring fibrils.
利用单克隆抗体GDA-J/F3,鉴定出了人皮肤基底膜带一种50 kDa的非胶原蛋白成分。用GDA-J/F3抗体对正常人皮肤进行免疫荧光染色,可见一条线性荧光标记基底膜带。免疫电镜显示,该表位定位于锚定原纤维插入致密板的位点。该抗原不同于胶原VII、锚定原纤维的主要结构蛋白以及基底膜带的其他几种结构分子,因为GDA-J/F3抗体在体外不与纯化的基底膜成分发生反应。在无血清培养中,该抗原由正常和转化的人角质形成细胞合成并分泌,正常人皮肤成纤维细胞的合成和分泌量较少。用GDA-J/F3抗体对放射性标记的上皮细胞条件培养基进行免疫沉淀,得到两条多肽,在非还原条件下,它们在SDS聚丙烯酰胺凝胶电泳上的表观分子量分别为46 kDa和50 kDa。使用还原凝胶时,仅观察到50 kDa的多肽。该抗原对细菌胶原酶消化有抗性,但对胰蛋白酶和胃蛋白酶敏感。它在低离子强度和碱性pH条件下也能与肝素和DEAE纤维素结合。这些发现表明,GDA-J/F3抗原是一种具有二硫键的小球形蛋白,有可能与基底膜蛋白聚糖相互作用。GDA-J/F3抗原整合到真皮-表皮连接处的组织结构中依赖于胶原VII的存在,因为在一些患有遗传性皮肤水疱病——营养不良性大疱性表皮松解症的患者中,GDA-J/F3表位缺失,这些患者缺乏胶原VII和锚定原纤维。