Zimmerli Simone C, Kerl Katrin, Hadj-Rabia Smail, Hohl Daniel, Hauser Conrad
Division of Immunology and Allergy, Department of Internal Medicine, Geneva University Hospitals and Medical School, Geneva, Switzerland.
Exp Dermatol. 2008 Jan;17(1):20-3. doi: 10.1111/j.1600-0625.2007.00624.x.
Claudin-1 (CLDN1) is a structural tight junction (TJ) protein and is expressed in differentiating keratinocytes and Langerhans cells in the epidermis. Our objective was to identify immunoreactive CLDN1 in human epidermal Langerhans cells and to examine the pattern of epidermal Langerhans cells in genetic human CLDN1 deficiency [neonatal ichthyosis, sclerosing cholangitis (NISCH) syndrome]. Epidermal cells from healthy human skin labelled with CLDN1-specific antibodies were analysed by confocal laser immunofluorescence microscopy and flow cytometry. Skin biopsy sections of two patients with NISCH syndrome were stained with an antibody to CD1a expressed on epidermal Langerhans cells. Epidermal Langerhans cells and a subpopulation of keratinocytes from healthy skin were positive for CLDN1. The gross number and distribution of epidermal Langerhans cells of two patients with molecularly confirmed NISCH syndrome, however, was not grossly altered. Therefore, CLDN1 is unlikely to play a critical role in migration of Langerhans cells (or their precursors) to the epidermis or their positioning within the epidermis. Our findings do not exclude a role of this TJ molecule once Langerhans cells have left the epidermis for draining lymph nodes.
闭合蛋白-1（CLDN1）是一种构成紧密连接（TJ）的蛋白质，在表皮中分化的角质形成细胞和朗格汉斯细胞中表达。我们的目的是在人类表皮朗格汉斯细胞中鉴定免疫反应性CLDN1，并研究遗传性人类CLDN1缺陷[新生儿鱼鳞病、硬化性胆管炎（NISCH）综合征]中表皮朗格汉斯细胞的模式。用CLDN1特异性抗体标记的健康人皮肤表皮细胞通过共聚焦激光免疫荧光显微镜和流式细胞术进行分析。两名NISCH综合征患者的皮肤活检切片用表皮朗格汉斯细胞上表达的CD1a抗体染色。健康皮肤的表皮朗格汉斯细胞和角质形成细胞亚群对CLDN1呈阳性。然而，两名经分子确诊的NISCH综合征患者的表皮朗格汉斯细胞的总数和分布没有明显改变。因此，CLDN1不太可能在朗格汉斯细胞（或其前体）迁移到表皮或它们在表皮内的定位中起关键作用。我们的研究结果并不排除这种TJ分子在朗格汉斯细胞离开表皮前往引流淋巴结后的作用。
