Hashimoto K, Fujiwara K, Tada J, Harada M, Setoyama M, Eto H
Department of Dermatology, Wayne State University School of Medicine, Detroit, MI 48201, USA.
J Cutan Pathol. 1995 Dec;22(6):488-501. doi: 10.1111/j.1600-0560.1995.tb01145.x.
Proteins involved in the formation of desmosomes and simpler adherens junctions were studied in three types of non-immune acantholytic diseases; specifically, four cases of Grover's disease (GD), one case of Hailey-Hailey's disease (HHD) and one case of Darier's disease (DD), and these were compared to two cases of immune-mediated acantholytic disease pemphigus vulgaris (PV). The proteins studied included: 1. The intracellular desmosomal proteins, desmoplakin I and II and plakoglobin; 2. The intercellular desmosomal proteins, desmoglein and CD44; and 3. vinculin, which is a major intracellular protein of the simpler aherens junctions. In GD, HHD and DD, immunostaining showed a loss of desmoplakin I and II and plakoglobin from the desmosomes, and a diffuse staining in the cytoplasm. In contrast, in pemphigus vulgaris, these proteins seemed intact and were localized to dot-like spots on the cell surface. Also, desmoglein, and CD44 were slightly affected in GD, and moderately affected in HHD and DD. Absence of desmosomal attachment plaques, the lack of labeling with desmoglein in the affected desmosomes and a diffusion of the labels into cytoplasm were demonstrated with electron microscopy using an immunogold technique. In PV, desmoglein III is one of the target antigens for the autoantibodies in this disease and was only partially preserved in a small number of lesional cells, while CD44 was mostly preserved. Vinculin was intact in GD, HHD and DD, but was lost in PV. This study, our previous work, and that of others, suggest that: 1. In GD, HHD and DD, the proteins of the desmosomal attachment plaque are primarily affected; 2. In PV, the intercellular glycoproteins are primarily involved; and 3. Simple adherens junctions are intact in GD, HHD and DD, but are damaged in PV.
在三种非免疫性棘层松解性疾病中，对参与桥粒和较简单黏着连接形成的蛋白质进行了研究；具体而言，研究了4例格罗弗病（GD）、1例黑利-黑利病（HHD）和1例达里埃病（DD），并将其与2例免疫介导的棘层松解性疾病寻常型天疱疮（PV）进行比较。所研究的蛋白质包括：1. 细胞内桥粒蛋白，桥粒斑蛋白I和II以及桥粒胶蛋白；2. 细胞间桥粒蛋白，桥粒芯糖蛋白和CD44；3. 纽蛋白，它是较简单黏着连接的主要细胞内蛋白。在GD、HHD和DD中，免疫染色显示桥粒斑蛋白I和II以及桥粒胶蛋白从桥粒中缺失，并在细胞质中呈弥漫性染色。相比之下，在寻常型天疱疮中，这些蛋白质似乎完好无损，并定位在细胞表面的点状斑点上。此外，桥粒芯糖蛋白和CD44在GD中受到轻微影响，在HHD和DD中受到中度影响。使用免疫金技术的电子显微镜显示，桥粒附着斑缺失，受累桥粒中桥粒芯糖蛋白标记缺乏，且标记物扩散到细胞质中。在PV中，桥粒芯糖蛋白III是该疾病自身抗体的靶抗原之一，仅在少数病变细胞中部分保留，而CD44大多保留。纽蛋白在GD、HHD和DD中完好无损，但在PV中缺失。这项研究、我们之前的工作以及其他人的研究表明：1. 在GD、HHD和DD中，桥粒附着斑的蛋白质主要受到影响；2. 在PV中，细胞间糖蛋白主要受累；3. 简单黏着连接在GD、HHD和DD中完好无损，但在PV中受损。
