Department of Dermatology, University of North Carolina, Chapel Hill, North Carolina 27599-7287, USA.
J Biol Chem. 2010 Mar 19;285(12):8936-41. doi: 10.1074/jbc.M109.087999. Epub 2010 Jan 21.
Pemphigus vulgaris (PV) is an autoimmune blistering disease in which antibodies against the desmosomal cadherin, DSG3 (desmoglein-3), cause acantholysis. It has become increasingly clear that loss of cell-cell adhesion in PV is a complex and active process involving multiple signaling events such as activation of p38MAPK. It has also been demonstrated that incubating keratinocytes with PV IgG causes a redistribution of DSG3 from the cell surface to endosomes, which target these proteins for degradation. This study was undertaken to determine the relationship between p38MAPK and DSG3 endocytosis in pemphigus. In this work, we confirm that PV IgG causes internalization of cell-surface DSG3 into endosomes (as early as 4 h), which are then depleted from both detergent-soluble and detergent-insoluble pools. Cell-surface DSG3 internalization and depletion from both the detergent-soluble and detergent-insoluble fractions were blocked by the p38MAPK inhibitor SB202190. These data suggest that p38MAPK is capable of regulating PV IgG-mediated DSG3 internalization and that previously isolated mechanistic observations may be linked to a common pathway by which pemphigus autoantibodies lead to acantholysis.
寻常型天疱疮(PV)是一种自身免疫性水疱病,其中针对桥粒钙黏蛋白 DSG3(桥粒芯糖蛋白 3)的抗体导致棘层松解。越来越明显的是,PV 中的细胞间黏附丧失是一个涉及多个信号事件的复杂和活跃过程,例如 p38MAPK 的激活。已经证明,将 PV IgG 孵育角质形成细胞会导致 DSG3 从细胞表面重新分布到内体,这些蛋白质随后被靶向降解。这项研究旨在确定寻常型天疱疮中 p38MAPK 和 DSG3 内吞作用之间的关系。在这项工作中,我们证实了 PV IgG 会导致细胞表面 DSG3 内吞到内体中(早在 4 小时),然后从洗涤剂可溶和不可溶部分中耗尽。p38MAPK 抑制剂 SB202190 阻断了细胞表面 DSG3 的内化以及洗涤剂可溶和不可溶部分的耗竭。这些数据表明,p38MAPK 能够调节 PV IgG 介导的 DSG3 内化,并且先前分离的机制观察结果可能与寻常型天疱疮自身抗体导致棘层松解的共同途径相关。