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天疱疮自身抗原桥粒芯糖蛋白1在人胸腺中的表达。

Expression of pemphigus-autoantigen desmoglein 1 in human thymus.

作者信息

Mouquet H, Berrih-Aknin S, Bismuth J, Joly P, Gilbert D, Tron F

机构信息

INSERM U905, Institut Fédératif de Recherche 23, Rouen, France.

出版信息

Tissue Antigens. 2008 May;71(5):464-70. doi: 10.1111/j.1399-0039.2008.01020.x. Epub 2008 Mar 10.

Abstract

Desmoglein (Dsg) 1 is a transmembrane glycoprotein of the desmosome allowing cell-cell adhesion between keratinocytes, whose expression is restricted to stratified squamous epithelia-like epidermis. Dsg1 is the target autoantigen of pathogenic autoantibodies produced by pemphigus foliaceus and 50% of pemphigus vulgaris patients in a Dsg1-specific T-cell-dependent pathway. Herewith, we show that mRNA of the DSG1 gene is present in normal human thymus and show by quantitative real-time polymerase chain reaction analysis that the expression of DSG1 transcript increases with age. Although immunoblot analysis on human thymus extracts using different anti-Dsg1 antibodies did not allow to detect the protein, we show by double-immunofluorescence assay that Dsg1 is expressed at protein level by CD19+ CD63+ cells located in the medulla. These data provide another illustration of the thymic expression of a tissue-specific autoantigen involved in an organ-specific autoimmune disease, which may participate in the tolerance acquisition and/or regulation of Dsg1-specific T cells.

摘要

桥粒芯糖蛋白(Dsg)1是桥粒的一种跨膜糖蛋白,可使角质形成细胞之间实现细胞间黏附,其表达仅限于分层鳞状上皮样表皮。Dsg1是落叶型天疱疮和50%寻常型天疱疮患者通过Dsg1特异性T细胞依赖途径产生的致病性自身抗体的靶自身抗原。在此,我们表明DSG1基因的mRNA存在于正常人类胸腺中,并通过定量实时聚合酶链反应分析表明DSG1转录本的表达随年龄增长而增加。尽管使用不同的抗Dsg1抗体对人胸腺提取物进行免疫印迹分析未能检测到该蛋白,但我们通过双重免疫荧光测定表明,位于髓质的CD19 + CD63 +细胞在蛋白水平表达Dsg1。这些数据为参与器官特异性自身免疫性疾病的组织特异性自身抗原的胸腺表达提供了另一个例证,这可能参与了Dsg1特异性T细胞的耐受性获得和/或调节。

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