Institute of Dermatology, School of Biosciences and Biotechnologies, University of Modena and Reggio Emilia, Via del Pozzo, 71 41100 Modena Italy.
Curr Pharm Biotechnol. 2012 Aug;13(10):1877-81. doi: 10.2174/138920112802273236.
Pemphigus is a group of rare autoimmune blistering diseases of the skin in which autoantibodies to desmosome cadherins, desmogleins, induce loss of cell-cell adhesion (acantholysis). In addition to steric hindrance and activation of intracellular phosphorylation cascade signaling pathways, apoptosis has been suggested to contribute to the mechanism by which pathogenic IgG induces acantholysis. We review the literature examining the role of apoptosis in pemphigus. Current data recognize a central role of apoptosis in the mechanisms of blister induction. In particular, here we stress the key role of FasL in pemphigus, as it is able to first induce apoptosis, then acantholysis. Being pro-apoptotic molecules important in blister formation, they could represent new specific targets for pemphigus treatment.
天疱疮是一组罕见的自身免疫性水疱性皮肤病,其中针对桥粒钙黏蛋白、桥粒芯糖蛋白的自身抗体导致细胞-细胞黏附丧失(棘层松解)。除了空间位阻和细胞内磷酸化级联信号通路的激活外,凋亡也被认为有助于致病性 IgG 诱导棘层松解的机制。我们综述了研究凋亡在天疱疮中的作用的文献。目前的数据认识到凋亡在水疱诱导机制中的核心作用。特别是,在这里我们强调 FasL 在天疱疮中的关键作用,因为它能够首先诱导凋亡,然后导致棘层松解。作为促凋亡分子在水疱形成中很重要,它们可能成为天疱疮治疗的新的特异性靶点。